1 Q Concerning PUK

circumferential  Autoimmune PUK is usually unilaterallaterality and sectoralextent . 2 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral 3 Concerning PUK

Autoimmune PUK 4 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral

improvement vs  It often heralds exacerbationworsening of systemic disease 5 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease 6 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease 7 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease 8 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid , Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so) 9 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? , Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so) 10 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so) 11 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so) 12 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK Ofpt doesthese not three, carry which a CTD/autoimmune is most likely to bediagnosis, associated what with should PUK? the ophthalmologist do? Initiate a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What percentage of PUK pts have RA as their underlying condition? 30-40

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 13 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK Ofpt doesthese not three, carry which a CTD/autoimmune is most likely to bediagnosis, associated what with should PUK? the ophthalmologist do? Initiate a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What percentage of PUK pts have RA as their underlying condition? 30-40

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 14 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK Ofpt doesthese not three, carry which a CTD/autoimmune is most likely to bediagnosis, associated what with should PUK? the ophthalmologist do? Initiate a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What percentage of PUK pts have RA as their underlying condition? 30-40

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 15 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK Ofpt doesthese not three, carry which a CTD/autoimmune is most likely to bediagnosis, associated what with should PUK? the ophthalmologist do? Initiate a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What percentage of PUK pts have RA as their underlying condition? Up to 40

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 16 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK Ofpt doesthese not three, carry which a CTD/autoimmune is most likely to bediagnosis, associated what with should PUK? the ophthalmologist do? Initiate a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What percentage of PUK pts have RA as their underlying condition? Up to 40

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 17 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK Ofpt doesthese not three, carry which a CTD/autoimmune is most likely to bediagnosis, associated what with should PUK? the ophthalmologist do? Initiate a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What percentage of PUK pts have RA as their underlying condition? Up to 40

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 18 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If aIs PUKPUK Ofthept doesthesemost commonnot three, carry which ocular a CTD/autoimmune ismanifestation most likely of to RA? bediagnosis, associated what with should PUK? the ophthalmologist do? InitiateNo a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What is,What then? percentage of PUK pts have RA as their underlying condition? KeratoconjunctivitisUp to 40 sicca

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 19 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If aIs PUKPUK Ofthept doesthesemost commonnot three, carry which ocular a CTD/autoimmune ismanifestation most likely of to RA? bediagnosis, associated what with should PUK? the ophthalmologist do? InitiateNo a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What is,What then? percentage of PUK pts have RA as their underlying condition? KeratoconjunctivitisUp to 40 sicca

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 20 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If aIs PUKPUK Ofthept doesthesemost commonnot three, carry which ocular a CTD/autoimmune ismanifestation most likely of to RA? bediagnosis, associated what with should PUK? the ophthalmologist do? InitiateNo a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What is,What then? percentage of PUK pts have RA as their underlying condition? KeratoconjunctivitisUp to 40 sicca

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 21 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If aIs PUKPUK Ofthept doesthesemost commonnot three, carry which ocular a CTD/autoimmune ismanifestation most likely of to RA? bediagnosis, associated what with should PUK? the ophthalmologist do? InitiateNo a RA,diagnostic by a substantial workup (or margin promptly arrange for a rheumatologist to do so)

What is,What then? percentage of PUK pts have RA as their underlying condition? KeratoconjunctivitisUp to 40 sicca

In addition to the peripheral cornea, what other ocular structure is commonly affected in these pts? The sclera 22 Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticThe term workup‘Wegener’s (or granupromptlylomatosis’ arrange has fallenfor a outrheumat of favor.ologist to do so) 23 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa ? If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticThe term workup‘Wegener’s (or granupromptlylomatosis’ arrange has fallenfor a outrheumat of favor.ologist to do so) What term is preferred in its place? 24 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa granulomatosis with polyangiitis If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticThe term workup‘Wegener’s (or granupromptlylomatosis’ arrange has fallenfor a outrheumat of favor.ologist to do so) What term is preferred in its place? ‘Granulomatosis with polyangiitis’ 25 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa granulomatosis with polyangiitis If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticThe term workup‘Wegener’s (or granulomatosis’promptly arrange has fallenfor a rheumatout of favor.ologist to do so) What term is preferred in its place? ‘GranulomatosisWhy with did polyangiitis’ the name ‘Wegener’s granulomatosis’ fall out of favor? Because Dr. Wegener was a Nazi 26 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, Wegener’s granulomatosis, and polyarteritis nodosa granulomatosis with polyangiitis If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticThe term workup‘Wegener’s (or granulomatosis’promptly arrange has fallenfor a rheumatout of favor.ologist to do so) What term is preferred in its place? ‘GranulomatosisWhy with did polyangiitis’ the name ‘Wegener’s granulomatosis’ fall out of favor? Because Dr. Wegener was a Nazi 27 Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK ptWhat does is notthe carryclassic a CTD/autoimmunetriad of granulomatosis diagnosis, with polyangiitis what should (GwP) the ophthalmologist? do? Necrotizing vasculitis of: Initiate a diagnostic workup (orIf promptly you’re having arrange trouble for rememberi a rheumatng ologistthat granulomatosis to do so) with --the upper and lowerpolyangiitis respiratory (GwP) tract is the entity formerly known as Wegener’s… --the kidneys --small and medium-sized arteries and veins 28 Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK ptWhat does is notthe carryclassic a CTD/autoimmunetriad of granulomatosis diagnosis, with polyangiitis what should (G thewP) ophthalmologist? do? Necrotizing vasculitis of: Initiate a diagnostic workup (orIf promptly you’re having arrange trouble for rememberi a rheumatng ologistthat granulomatosis to do so) with --the upper and lowerpolyangiitis respiratory (GwP) tract is the entity formerly known as Wegener’s… --the kidneys Think of the little ‘w’ as standing for ‘Wegener’s’ --small and medium-sized arteries and veins 29 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK ptWhat does is notthe carryclassic a CTD/autoimmunetriad of granulomatosis diagnosis, with polyangiitis what should (GwP)? the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --the upper and lower respiratory tract --the kidneys --small and medium-sized arteries and veins 30 Q/A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK ptWhat does is notthe carryclassic a CTD/autoimmunetriad of granulomatosis diagnosis, with polyangiitis what should (GwP)? the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --the upper and lower respiratory tract --the kidneys --small and medium-sized arteries and veins 31 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK ptWhat does is notthe carryclassic a CTD/autoimmunetriad of granulomatosis diagnosis, with polyangiitis what should (GwP)? the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --the upper and lower respiratory tract --the kidneys --small and medium-sized arteries and veins 32 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK ptWhat does is notthe carryclassic a CTD/autoimmunetriad of granulomatosis diagnosis, with polyangiitis what should (GwP)? the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or promptly of: arrange for a rheumatologist to do so) --the upper and lower respiratory tract? --the kidneys? --small and medium-sized arteries and veins?

What is the classic manifestation of the classic triad? That is, with what specific condition do these pts always present?*

*On the OKAP and Boards, that is 33 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK ptWhat does is notthe carryclassic a CTD/autoimmunetriad of granulomatosis diagnosis, with polyangiitis what should (GwP)? the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or promptly of: arrange for a rheumatologist to do so) --the upper and lower respiratory tract --the kidneys --small and medium-sized arteries and veins

What is the classic manifestation of the classic triad? That is, with what specific condition do these pts always present?* Sinusitis. Don’t diagnose a pt with GwP without it!*

*On the OKAP and Boards, that is 34 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK)

Is retinal involvement in GwP a thing? Yes, albeit uncommonly 35 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK)

Is retinal involvement in GwP a thing? Yes, albeit uncommonly 36 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK)

Is retinal involvement in GwP a thing? Yes, albeit uncommonly 37 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK)

Is retinal involvement in GwP a thing? Yes, albeit uncommonly 38 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK)

Is retinal involvement in GwP a thing? Yes, albeit uncommonly 39 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK)

Is retinal involvement in GwP a thing? Yes, albeit uncommonly 40 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK)

Is retinal involvement in GwP a thing? Yes, albeit uncommonly 41 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK)

Is retinal involvement in GwP a thing? Yes, albeit uncommonly 42 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK) What form does retinal involvement in GwP typically take? Is retinal involvement in GwPA retinal a thing? vasculitis Yes, albeit uncommonly 43 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK) What form does retinal involvement in GwP typically take? Is retinal involvement in GwPA retinal a thing? vasculitis (not surprisingly) Yes, albeit uncommonly 44 Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizing workup vasculitis (or of:promptly arrange for a rheumatologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK) What form does retinal involvement in GwP typically take? Is retinal involvement in GwPA retinal a thing? vasculitis (not surprisingly) Yes, albeit uncommonly 45 Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

WhatWhat proportionis the classic of GwP triad pts of have granul ophthalmicomatosis involvement? with polyangiitis (GwP)? If a PUK ptAbout does half not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnosticNecrotizingFor moreworkup vasculitis (oron of:promptly GwP, arrange see slide-set for a rheumat U1ologist to do so) --theWhat upper is the mostand commonlower respiratory manifestation tract of that involvement? (It’s not PUK.) --theOrbital kidneys inflammation --small and medium-sized arteries and veins What is the next most common manifestation? Scleritis (including PUK) What form does retinal involvement in GwP typically take? Is retinal involvement in GwPA retinal a thing? vasculitis (not surprisingly) Yes, albeit uncommonly 46 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD ) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 47 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 48 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 49 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 50 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 51 Q/A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA maleM a v Fdiagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 52 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 53 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 54 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 55 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 56 Q/A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 57 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPAN pt?a CTD/autoimmune diagnosis, what should the ophthalmologist do? InitiateA male a diagnostic between workup 40 and (or 60 promptlyyears old arrange for a rheumatologist to do so)

Is there a racial predilection? No

PAN is strongly associated with seropositivity for what virus? Hepatitis B 58 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPANWhat pt?a is CTD/autoimmune the typical presentation diagnosis, of PAN? what(Note: should It’s not theophthalmic.) ophthalmologist do? InitiateA male a diagnostic between workup 40Constitutional and (or 60 promptlyyears symptoms: old arrange Fever, for fatigue, a rheumat weightologist loss. Renal to do vas so)culitis resulting in secondary HTN is common. Is there a racial predilection? No What proportion of PAN pts develop ophthalmic involvement? About 20% PAN is strongly associated with seropositivity for what virus? Hepatitis B 59 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPANWhat pt?a is CTD/autoimmune the typical presentation diagnosis, of PAN? what(Note: should It’s not theophthalmic.) ophthalmologist do? InitiateA male a diagnostic between workup 40Constitutional and (or 60 promptlyyears symptoms: old arrange Fever, for fatigue, a rheumat weightologist loss. Renal to do vas so)culitis resulting in secondary HTN is common. Is there a racial predilection? No What proportion of PAN pts develop ophthalmic involvement? About 20% PAN is strongly associated with seropositivity for what virus? Hepatitis B 60 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPANWhat pt?a is CTD/autoimmune the typical presentation diagnosis, of PAN? what(Note: should It’s not theophthalmic.) ophthalmologist do? InitiateA male a diagnostic between workup 40Constitutional and (or 60 promptlyyears symptoms: old arrange Fever, for fatigue, a rheumat weightologist loss. Renal to do vas so)culitis resulting in secondary HTN is common. Is there a racial predilection? No What proportion of PAN pts develop ophthalmic involvement? About 20% PAN is strongly associated with seropositivity for what virus? Hepatitis B 61 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

In a nutshell, what is the pathophysiology of PAN? WithSubacute which connective-tissue episodes of focal diseases necrotizing (CTD inflammations) and/or vasculitides has PUK been associated? Prettyof arteries much all of them

WhichIs it athree common, conditions or uncommon are most likelycondition? to present with PUK? RheumatoidUncommon arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If aWho PUK is pt the does typical not carryPANWhat pt?a is CTD/autoimmune the typical presentation diagnosis, of PAN? what(Note: should It’s not theophthalmic.) ophthalmologist do? InitiateA male a diagnostic between workup 40Constitutional and (or 60 promptlyyears symptoms: old arrange Fever, for fatigue, a rheumat weightologist loss. Renal to do vas so)culitis resulting in secondary HTN is common. Is there a racial predilection? No What proportion of PAN pts develop ophthalmic involvement? About 20% PAN is strongly associated with seropositivity for what virus? Hepatitis B 62 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so) 63 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so) 64 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so)

If the workup is negative, what non-autoimmune diagnosis should you consider? Infectious PUK

If the PUK is associated with copious mucopurulent discharge, what infectious etiology should you consider? Gonococcal 65 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so)

If the workup is negative, what non-autoimmune diagnosis should you consider? Infectious PUK

If the PUK is associated with copious mucopurulent discharge, what infectious etiology should you consider? Gonococcal 66 Q Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so)

If the workup is negative, what non-autoimmune diagnosis should you consider? Infectious PUK

If the PUK is associated with copious mucopurulent discharge, what infectious etiology should you consider? Gonococcal 67 A Concerning PUK

With what general category of autoimmune dz is PUK associated?  Autoimmune PUKConnective-tissue is usually dz, unilateral especially vasculitides and sectoral  It often heralds exacerbation of systemic disease

With which connective-tissue diseases (CTDs) and/or vasculitides has PUK been associated? Pretty much all of them

Which three conditions are most likely to present with PUK? Rheumatoid arthritis, granulomatosis with polyangiitis, and polyarteritis nodosa

If a PUK pt does not carry a CTD/autoimmune diagnosis, what should the ophthalmologist do? Initiate a diagnostic workup (or promptly arrange for a rheumatologist to do so)

If the workup is negative, what non-autoimmune diagnosis should you consider? Infectious PUK

If the PUK is associated with copious mucopurulent discharge, what infectious etiology should you consider? Gonococcal disease 68 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL,4 treatment patching maneuvers  You should also consider stopping topical steroids, which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, avoid topical steroids. 3) Suppress systemic inflammation 69 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue  You should also consider stopping topical steroids, which can delay re- epithelialization. As a general rule: If the cornea(bandage iscontact signific lens) antly thinned, avoid topical steroids. 3) Suppress systemic inflammation 70 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting Of these three maneuvers, which is paramount? 2) Promote re-epithelialization viaControlling lubes, BCL,the underlying patching, disease glue process--  You should also consider stoppingwithout topical this, steroids,the other maneuverswhich can aredelay akin re- to re- epithelialization. As a general rule:arranging If the thecornea deck ischairs signific on antlythe Titanic thinned, avoid topical steroids. 3) Suppress systemic inflammation 71 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting Of these three maneuvers, which is paramount? 2) Promote re-epithelialization via Controllinglubes, BCL, the underlyingpatching, disease glue process--  You should also consider stoppingwithout topical this, steroids,the other maneuverswhich can aredelay akin re- to re- epithelialization. As a general rule:arranging If the thecornea deck ischairs signific on antlythe Titanic thinned, avoid topical steroids. 3) Suppress systemic inflammation 72 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue How should one improve wetting? With frequent You shoulddosing ofalso preservative-free consider stopping artificial topical tears (PFsteroids, ATs) which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, In additionavoid to improving topical steroids. wetting, what other benefit derives from frequent PF AT use? They3) Suppress will remove systemicinflammatory inflammation cytokines from ifthe local ocular maneuvers surface are 73 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue How should one improve wetting? With frequent You shoulddosing ofalso preservative-free consider stopping artificial topical tears (PFsteroids, ATs) which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, In additionavoid to improving topical steroids. wetting, what other benefit derives from frequent PF AT use? They3) Suppress will remove systemicinflammatory inflammation cytokines from ifthe local ocular maneuvers surface are 74 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue How should one improve wetting? With frequent You shoulddosing ofalso preservative-free consider stopping artificial topical tears (PFsteroids, ATs) which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, In additionavoid to improving topical steroids. wetting, what other benefit derives from frequent PF AT use? They3) Suppress will remove systemicinflammatory inflammation cytokines from ifthe local ocular maneuvers surface are 75 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue How should one improve wetting? With frequent You shoulddosing ofalso preservative-free consider stopping artificial topical tears (PFsteroids, ATs) which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, In additionavoid to improving topical steroids. wetting, what other benefit derives from frequent PF AT use? They3) Suppress will remove systemicinflammatory inflammation cytokines from ifthe local ocular maneuvers surface are 76 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma 77 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma 78 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma 79 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma 80 Concerning PUK

Just prior to perfing Same eye s/p gluing (and on IMT)

PUK in RA 81 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma

Use of cyanoacrylate adhesive mandates that what other therapeutic maneuver be applied as well? A BCL must be placed over the glued cornea 82 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma

Use of cyanoacrylate adhesive mandates that what other therapeutic maneuver be applied as well? A BCL must be placed over the glued cornea 83 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma

Use of cyanoacrylate adhesive mandates that what other therapeutic maneuver be applied as well? A BCL must be placed over the glued cornea Use of a BCL mandates that what other therapeutic maneuver be applied as well? An antibiotic drop should be used to prophylax against the possibility of a BCL-induced bacterial superinfection 84 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma

Use of cyanoacrylate adhesive mandates that what other therapeutic maneuver be applied as well? A BCL must be placed over the glued cornea Use of a BCL mandates that what other therapeutic maneuver be applied as well? An antibiotic drop should be used to prophylax against the possibility of a BCL-induced bacterial superinfection 85 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma

Use of cyanoacrylate adhesive mandates that what other therapeutic maneuver be applied as well? A BCL must be placed over the glued cornea Use of a BCL mandates that what other therapeutic maneuver be applied as well? An antibiotic drop should be used to prophylax against the possibility of a BCL-induced bacterial superinfection

What bacteria species must you be certain is adequately covered by the antibiotic? Pseudomonas 86 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue What specificYou should sort of gluealso is consider being referred stopping to here? topical steroids, which can delay re- Cyanoacrylateepithelialization. adhesive As a general rule: If the cornea is significantly thinned, avoid topical steroids. How does glue assist in PUK healing? 1)3) It Suppressprovides tectonic systemic stability inflammation, thereby reducing the risk of perforation 2) It acts as a barrier preventing PMNs from reaching (and destroying) corneal stroma

Use of cyanoacrylate adhesive mandates that what other therapeutic maneuver be applied as well? A BCL must be placed over the glued cornea Use of a BCL mandates that what other therapeutic maneuver be applied as well? An antibiotic drop should be used to prophylax against the possibility of a BCL-induced bacterial superinfection

What bacteria species must you be certain is adequately covered by the antibiotic? Pseudomonas 87 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue common ocular drug  You should also consider stopping topical steroids, which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, avoid topicalsame steroids. drug 3) Suppress systemic inflammation 88 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue  You should also consider stopping topical steroids, which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, avoid topical steroids. 3) Suppress systemic inflammation 89 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue  You should also consider stopping topical steroids, which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, avoid topical steroids. 3) Suppress systemic inflammation 4) Conj flap over the peripheral defect? What about using a conj flap to cover the peripheral defect? Conj flaps are contraindicated in autoimmune PUK because they bring the conj vasculature (and thus all those nasty blood-borne inflammatory mediators) even closer to the melt

What conj surgery is very helpful in autoimmune PUK? Sectoral conj resection (ie, cutting the conj away from the PUK zone) can be very effective 90 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue  You should also consider stopping topical steroids, which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, avoid topical steroids. 3) Suppress systemic inflammation 4) Conj flap over the peripheral defect? NO! What about using a conj flap to cover the peripheral defect? Conj flaps are contraindicated in autoimmune PUK because they bring the conj vasculature (and thus all those nasty blood-borne inflammatory mediators) even closer to the melt

What conj surgery is very helpful in autoimmune PUK? Sectoral conj resection (ie, cutting the conj away from the PUK zone) can be very effective 91 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue  You should also consider stopping topical steroids, which can delay re- In what clinicalepithelialization. scenario might Asa conj a general flap over a rule: PUK defectIf the be cornea an appropri is significate treatmentantly maneuver? thinned, In infectiousavoidPUK, topical especially steroids. when the organism is fungal 3) Suppress systemic inflammation 4) Conj flap over the peripheral defect? YES! What about using a conj flap to cover the peripheral defect? Conj flaps are contraindicated in autoimmune PUK because they bring the conj vasculature (and thus all those nasty blood-borne inflammatory mediators) even closer to the melt

What conj surgery is very helpful in autoimmune PUK? Sectoral conj resection (ie, cutting the conj away from the PUK zone) can be very effective 92 A/Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue  You should also consider stopping topical steroids, which can delay re- In what clinicalepithelialization. scenario might Asa conj a general flap over a rule: PUK defectIf the be cornea an appropri is significate treatmentantly maneuver? thinned, In infectiousavoidPUK, topical especially steroids. when the organism is fungaltype of bug 3) Suppress systemic inflammation 4) Conj flap over the peripheral defect? YES! What about using a conj flap to cover the peripheral defect? Conj flaps are contraindicated in autoimmune PUK because they bring the conj vasculature (and thus all those nasty blood-borne inflammatory mediators) even closer to the melt

What conj surgery is very helpful in autoimmune PUK? Sectoral conj resection (ie, cutting the conj away from the PUK zone) can be very effective 93 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue  You should also consider stopping topical steroids, which can delay re- In what clinicalepithelialization. scenario might Asa conj a generalflap over a rule: PUK defectIf the be cornea an appropri is significate treatmentantly maneuver? thinned, In infectiousavoidPUK, topical especially steroids. when the organism is fungal 3) Suppress systemic inflammation 4) Conj flap over the peripheral defect? YES! What about using a conj flap to cover the peripheral defect? Conj flaps are contraindicated in autoimmune PUK because they bring the conj vasculature (and thus all those nasty blood-borne inflammatory mediators) even closer to the melt

What conj surgery is very helpful in autoimmune PUK? Sectoral conj resection (ie, cutting the conj away from the PUK zone) can be very effective 94 Q Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue  You should also consider stopping topical steroids, which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, avoid topical steroids. 3) Suppress systemic inflammation 4) Conj surgery: What about using a conj flap to cover the peripheral defect? Conj flaps are contraindicated in autoimmune PUK because they bring the conj vasculature (and thus all those nasty blood-borne inflammatory mediators) even closer to the melt

What conj surgery is very helpful in autoimmune PUK? Sectoral conj resection (ie, cutting the conj away from the PUK zone) can be very effective 95 A Concerning PUK

 Autoimmune PUK is usually unilateral and sectoral  It often heralds exacerbation of systemic disease  The treatment goal is to stop K melting through 3 maneuvers: 1) Improve wetting 2) Promote re-epithelialization via lubes, BCL, patching, glue  You should also consider stopping topical steroids, which can delay re- epithelialization. As a general rule: If the cornea is significantly thinned, avoid topical steroids. 3) Suppress systemic inflammation 4) Conj surgery: Sectoral conj resection What about using a conj flap to cover the peripheral defect? Conj flaps are contraindicated in autoimmune PUK because they bring the conj vasculature (and thus all those nasty blood-borne inflammatory mediators) even closer to the melt

What conj surgery is very helpful in autoimmune PUK? Sectoral conj resection (ie, cutting the conj away from the PUK zone) can be very effective 96 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): 97 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP 98 Concerning PUK

Saddle-nose deformity 99 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP

If a pt with a saddle nose had interstitial keratitis rather than PUK, what diagnosis should you consider? 100 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP

If a pt with a saddle nose had interstitial keratitis rather than PUK, what diagnosis should you consider? Congenital 101 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: 102 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS 103 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: 104 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP 105 Concerning PUK

Auricular damage in RP 106 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): 107 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN 108 Concerning PUK

Mooren’s ulcer. Note the overhanging edge. 109 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN

What is the classic description regarding the pattern of progression for PUK in both Mooren’s and PAN? 110 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A/QPolyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN

What is the classic description regarding the pattern of progression for PUK in both Mooren’s and PAN? 1) Starts sectoral, then 2) progresses circumferentially, then 3) progresses centrally 111 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN

What is the classic description regarding the pattern of progression for PUK in both Mooren’s and PAN? 1) Starts sectoral, then 2) progresses circumferentially, then 3) progresses centrally 112 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: 113 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU 114 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU

Take note! This is a key factor differentiating between Mooren’s and other forms of PUK. 115 Concerning PUK

Mooren’s ulcer. Note the adjacent sclera is totally quiet 116 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): 117 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS (Hey, what about PAN??!! Un momento, por favor) 118 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS

What does ANCA stand for? 119 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS

What does ANCA stand for? Antineutrophil cytoplasmic antibodies 120 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS

What does ANCA stand for? Antineutrophil cytoplasmic antibodies

What are they? 121 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS

What does ANCA stand for? Antineutrophil cytoplasmic antibodies

What are they? Autoantibodies against antigens found within the cytoplasm of neutrophils 122 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS

What does ANCA stand for? Antineutrophil cytoplasmic antibodies

What are they? Autoantibodies against antigens found within the cytoplasm of neutrophils

With which specific ANCA pattern is each condition associated? Granulomatosis with polyangiitis: Churg-Strauss: 123 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS

What does ANCA stand for? Antineutrophil cytoplasmic antibodies

What are they? Autoantibodies against antigens found within the cytoplasm of neutrophils

With which specific ANCA pattern is each condition associated? Granulomatosis with polyangiitis: Cytoplasmic (c-ANCA) Churg-Strauss: Perinuclear (p-ANCA) 124 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN?

What about PAN? I thought it was ANCA-positive as well. 125 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A/QPolyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN?

What about PAN? I thought it was ANCA-positive as well. This is a sticky widget. In the 1990s, rheumatologists determined that the label PAN was being applied to conditions that were actually separate disease entities. Thus, PAN was subdivided into several conditions: --Classicword + abb.PAN, which affects only medium- and small-sized ‘muscular’ arteries; and --Microscopictwo words polyangiitis, which affects smaller arteries, arterioles, capillaries and venules. 126 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN?

What about PAN? I thought it was ANCA-positive as well. This is a sticky widget. In the 1990s, rheumatologists determined that the label PAN was being applied to conditions that were actually separate disease entities. Thus, PAN was subdivided into several conditions: --Classic PAN, which affects only medium- and small-sized ‘muscular’ arteries; and --Microscopic polyangiitis, which affects smaller arteries, arterioles, capillaries and venules. 127 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN?

What about PAN? I thought it was ANCA-positive as well. This is a sticky widget. In the 1990s, rheumatologists determined that the label PAN was being applied to conditions that were actually separate disease entities. Thus, PAN was subdivided into several conditions: --Classic PAN, which affects only medium- and small-sized ‘muscular’ arteries; and --Microscopic polyangiitis, which affects smaller arteries, arterioles, capillaries and venules. It turns out microscopic polyangiitis is strongly ANCA-positive, but classic PAN is not. (Because of its ANCA- positivity, microscopic angiitis is now considered to be more closely related to Churg-Strauss, and especially granulomatosis with polyangiitis, than it is to PAN). 128 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN?

What about PAN? I thought it was ANCA-positive as well. This is a sticky widget. In the 1990s, rheumatologists determined that the label PAN was being applied to conditions that were actually separate disease entities. Thus, PAN was subdivided into several conditions: --Classic PAN, which affects only medium- and small-sized ‘muscular’ arteries; and --Microscopic polyangiitis, which affects smaller arteries, arterioles, capillaries and venules. It turns out microscopic polyangiitis is strongly ANCA-positive, but classic PAN is not. (Because of its ANCA- positivity, microscopic What’sangiitis the is now difference considered between to be a more‘small-sized’ closely arteryrelated and to Churg-Strauss,a ‘smaller’ artery? and especially granulomatosis with polyangRule ofiitis, thumb: than Classic it is to PAN).PAN only affects arteries large enough to be named, whereas microscopic angiitis only affects vessels smaller than that. 129 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN?

What about PAN? I thought it was ANCA-positive as well. This is a sticky widget. In the 1990s, rheumatologists determined that the label PAN was being applied to conditions that were actually separate disease entities. Thus, PAN was subdivided into several conditions: --Classic PAN, which affects only medium- and small-sized ‘muscular’ arteries; and --Microscopic polyangiitis, which affects smaller arteries, arterioles, capillaries and venules. It turns out microscopic polyangiitis is strongly ANCA-positive, but classic PAN is not. (Because of its ANCA- positivity, microscopic What’sangiitis the is now difference considered between to be a more‘small-sized’ closely arteryrelated and to Churg-Strauss,a ‘smaller’ artery? and especially granulomatosis with polyangRule ofiitis, thumb: than Classic it is to PAN).PAN only affects arteries large enough to be named, whereas microscopic angiitis only affects vessels smaller than that. 130 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN?

What about PAN? I thought it was ANCA-positive as well. This is a sticky widget. In the 1990s, rheumatologists determined that the label PAN was being applied to conditions that were actually separate disease entities. Thus, PAN was subdivided into several conditions: --Classic PAN, which affects only medium- and small-sized ‘muscular’ arteries; and --Microscopic polyangiitis, which affects smaller arteries, arterioles, capillaries and venules. It turns out microscopic polyangiitis is strongly ANCA-positive, but classic PAN is not. (Because of its ANCA- positivity, microscopic angiitis is now considered to be more closely related to Churg-Strauss, and especially granulomatosis with polyangiitis, than it is to PAN).

Got it. So if it’s ANCA+ it’s not PAN, right? 131 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN (10% of cases)

What about PAN? I thought it was ANCA-positive as well. This is a sticky widget. In the 1990s, rheumatologists determined that the label PAN was being applied to conditions that were actually separate disease entities. Thus, PAN was subdivided into several conditions: --Classic PAN, which affects only medium- and small-sized ‘muscular’ arteries; and --Microscopic polyangiitis, which affects smaller arteries, arterioles, capillaries and venules. It turns out microscopic polyangiitis is strongly ANCA-positive, but classic PAN is not. (Because of its ANCA- positivity, microscopic angiitis is now considered to be more closely related to Churg-Strauss, and especially granulomatosis with polyangiitis, than it is to PAN).

Got it. So if it’s ANCA+ it’s not PAN, right? Unfortunately, no. Per the BCSC Uveitis book, ~10% of PAN pts will be c- or p-ANCA positive. 132 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN (10% of cases)

What about PAN? I thought it was ANCA-positive as well. This is a sticky widget. In the 1990s, rheumatologists determined that the label PAN was being applied to conditions that were actually separateOK, tell disease me this entities.much at Thus,least--are PAN both was classicsubdivided PAN into and several conditions: --Classic PAN, which affects onlymicroscopic medium- angiitis and small-sized associated ‘muscular’ with PUK? arteries; and --Microscopic polyangiitis, whichYes affects smaller arteries, arterioles, capillaries and venules. It turns out microscopic polyangiitis is strongly ANCA-positive, but classic PAN is not. (Because of its ANCA- positivity, microscopic angiitis is now considered to be more closely related to Churg-Strauss, and especially granulomatosis with polyangiitis, than it is to PAN).

Got it. So if it’s ANCA+ it’s not PAN, right? Unfortunately, no. Per the BCSC Uveitis book, ~10% of PAN pts will be c- or p-ANCA positive. 133 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN (10% of cases)

What about PAN? I thought it was ANCA-positive as well. This is a sticky widget. In the 1990s, rheumatologists determined that the label PAN was being applied to conditions that were actually separateOK, tell disease me this entities.much at Thus,least--are PAN both was classicsubdivided PAN into and several conditions: --Classic PAN, which affects onlymicroscopic medium- angiitis and small-sized associated ‘muscular’ with PUK? arteries; and --Microscopic polyangiitis, whichYes affects smaller arteries, arterioles, capillaries and venules. It turns out microscopic polyangiitis is strongly ANCA-positive, but classic PAN is not. (Because of its ANCA- positivity, microscopic angiitis is now considered to be more closely related to Churg-Strauss, and especially granulomatosis with polyangiitis, than it is to PAN).

Got it. So if it’s ANCA+ it’s not PAN, right? Unfortunately, no. Per the BCSC Uveitis book, ~10% of PAN pts will be c- or p-ANCA positive. 134 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: 135 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU 136 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): 137 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP 138 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: 139 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN 140 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN

Which hepatitis virus is definitely associated with PAN? 141 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN

Which hepatitis virus is definitely associated with PAN? Hepatitis B 142 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN

Which hepatitis virus is definitely associated with PAN? Hepatitis B

What percent of PAN pts test positive for are Hep B surface Ag ? About 10 143 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN

Which hepatitis virus is definitely associated with PAN? Hepatitis B

What percent of PAN pts test positive for are Hep B surface Ag ? About 10 144 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN

Which hepatitis virus is definitely associated with PAN? Hepatitis B

Which form is probably associated, but the evidence is not as strong as for B? 145 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN

Which hepatitis virus is definitely associated with PAN? Hepatitis B

Which form is probably associated, but the evidence is not as strong as for B? Hepatitis C 146 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN  Associated with helminthic seropositivity: 147 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN  Associated with helminthic seropositivity: MU 148 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN  Associated with helminthic seropositivity: MU  Renal function may be impaired (4): 149 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN  Associated with helminthic seropositivity: MU  Renal function may be impaired (4): GwP; PAN; CS; RP 150 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN  Associated with helminthic seropositivity: MU  Renal function may be impaired (4): GwP; PAN; CS; RP  Chronic, tx-resistant sinusitis common: 151 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN  Associated with helminthic seropositivity: MU  Renal function may be impaired (4): GwP; PAN; CS; RP  Chronic, tx-resistant sinusitis common: GwP 152 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) Q Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN  Associated with helminthic seropositivity: MU  Renal function may be impaired (4): GwP; PAN; CS; RP  Chronic, tx-resistant sinusitis common: GwP  Extremely painful: 153 For each statement, identify which of these causes of PUK is/are associated (some will more than one answer) A Polyarteritis nodosa (PAN) Relapsing polychondritis (RP) Rheumatoid arthritis (RA) Granulomatosis with polyangiitis (GwP) Mooren’s ulcer (MU) Churg-Strauss (CS)

 Saddle-nose deformity (2): RP; GwP  Asthma and eosinophilia: CS  Deformed auricular pinnae: RP  Ulcer has overhanging edge (2): MU; PAN  Sclera never involved: MU  ANCA positive (2): GwP; CS; PAN  Is a diagnosis of exclusion: MU  Chest X-ray likely abnormal (3): GwP; CS; RP  Associated with hepatitis seropositivity: PAN  Associated with helminthic seropositivity: MU  Renal function may be impaired (4): GwP; PAN; CS; RP  Chronic, tx-resistant sinusitis common: GwP  Extremely painful: MU (All forms of inflammatory PUK are painful, but Mooren’s is exceptionally so.) 154 Q Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD (IBD = Inflammatory bowel disease) 155 A Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD 156 Q Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

Why is Mooren’s the oddball in this group? 157 A Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

Why is Mooren’s the oddball in this group? PUK in the others is due to a systemic condition, whereas Mooren’s is, by definition, ocular only 158 Q Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE 159 A Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE 160 Q Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? 161 Q/A Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory -- 162 A Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory -- 163 Q/A Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 164 A Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 165 Q Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE

WhyIf the is epitheliumTerrien’s theis intact, oddball what in is this going group? on that puts Terrien’s on the DDx for PUK? TwoProgressive reasons: peripheral stromal thinning makes the limbal region in Terrien’s resemble PUK --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 166 Q/A Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE

WhyIf the is epitheliumTerrien’s theis intact, oddball what in is this going group? on that puts Terrien’s on the DDx for PUK? TwoProgressive reasons: peripheral stromal thinning makes the limbal region in Terrien’s resemble PUK --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 167 A Concerning PUK

 With respect to manifesting PUK, which of the following doesn’t belong, and why?  RA, Mooren’s, Behçet, IBD

 And in this group?  Mooren’s, Terrien’s marginal, Sarcoid, SLE

WhyIf the is epitheliumTerrien’s theis intact, oddball what in is this going group? on that puts Terrien’s on the DDx for PUK? TwoProgressive reasons: peripheral stromal thinning makes the limbal region in Terrien’s resemble PUK --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 168 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 169 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 170 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 171 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 172 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral,During orwhat bilateral? life-stage doesn’t does Terrien’s belong, typically first and appear? why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 173 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral,During orwhat bilateral? life-stage doesn’t does Terrien’s belong, typically first and appear? why? BilateralYoung (although adulthood involvement (late teens can be- early strikingly 30s) asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 174 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 175 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 176 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 177 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 178 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 179 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

Does it have a gender predilection?  WithWhile respect once thought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral, or bilateral? doesn’t belong, and why? Bilateral (although involvement can be strikingly asymmetric)  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 180 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

The keyDoes to diagnosing it have a gender Terrien’s predilection? is its classic appearance:  While once thought to be more common in males, it is now considered equal --TheWith epithelium respect is intact to, as manifestingnoted earlier PUK, which of --The leading edge of the thinned area is characterized by the presence of…Lipid theIs followingit unilateral, or bilateral? doesn’t belong, and why? --The trailingBilateral portion(although is involvement characterized can be by strikingly the presence asymmetric) of… A vascular pannus  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 181 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

The keyDoes to diagnosing it have a gender Terrien’s predilection? is its classic appearance:  While once thought to be more common in males, it is now considered equal --TheWith epithelium respect is intact to, as manifestingnoted earlier PUK, which of --The leading edge of the thinned area is characterized by the presence of…Lipid theIs followingit unilateral, or bilateral? doesn’t belong, and why? --The trailingBilateral portion(although is involvement characterized can be by strikingly the presence asymmetric) of… A vascular pannus  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 182 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

The keyDoes to diagnosing it have a gender Terrien’s predilection? is its classic appearance:  While once thought to be more common in males, it is now considered equal --TheWith epithelium respect is intact to, as manifestingnoted earlier PUK, which of --The leading edge of the thinned area is characterized by the presence of…Lipid theIs followingit unilateral, or bilateral? doesn’t belong, and why? --The trailingBilateral portion(although is involvement characterized can be by strikingly the presence asymmetric) of… A vascular pannus  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 183 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

The keyDoes to diagnosing it have a gender Terrien’s predilection? is its classic appearance:  While once thought to be more common in males, it is now considered equal --TheWith epithelium respect is intact to, as manifestingnoted earlier PUK, which of --The leading edge of the thinned area is characterized by the presence of…Lipid theIs followingit unilateral, or bilateral? doesn’t belong, and why? --The trailingBilateral portion(although is involvement characterized can be by strikingly the presence asymmetric) of… A vascular pannus  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 184 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

The keyDoes to diagnosing it have a gender Terrien’s predilection? is its classic appearance:  While once thought to be more common in males, it is now considered equal --TheWith epithelium respect is intact to, as manifestingnoted earlier PUK, which of --The leading edge of the thinned area is characterized by the presence of…Lipid theIs followingit unilateral, or bilateral? doesn’t belong, and why? --The trailingBilateral portion(although is involvement characterized can be by strikingly the presence asymmetric) of a…Vasculartwo words pannus  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 185 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

The keyDoes to diagnosing it have a gender Terrien’s predilection? is its classic appearance:  While once thought to be more common in males, it is now considered equal --TheWith epithelium respect is intact to, as manifestingnoted earlier PUK, which of --The leading edge of the thinned area is characterized by the presence of…Lipid theIs followingit unilateral, or bilateral? doesn’t belong, and why? --The trailingBilateral portion(although is involvement characterized can be by strikingly the presence asymmetric) of a…Vascular pannus  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally, then spreads circumferentially

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 186 Concerning PUK

Terrien marginal degeneration. Note the leading lipids and the trailing pannus 187 Concerning PUK

Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon

The keyDoes to diagnosing it have a gender Terrien’s predilection? is its classic appearance:  While once thought to be more common in males, it is now considered equal --TheWith epithelium respect is intact to, as manifestingnoted earlier PUK, which of --The leading edge of the thinned area is characterized by the presence of…Lipid theIs followingit unilateral, or bilateral? doesn’t belong, and why? --The trailingBilateral portion(although is involvement characterized can be by strikingly the presence asymmetric) of a…Vascular pannus  RA, Mooren’s, Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? RememberIt starts superonasally, this!!! then Consider spreads circumferentially it your tl;dr for Terrien’s

Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 188 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon Does Terrien’s render the cornea significantly thinner Does it havethan a gender normal? predilection?  WithWhile respect once Yesthought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral,Is the or bilateral?thinned doesn’t Terrien’s cornea belong, at risk for rupture and with why? Bilateral (althoughmild trauma? involvement can be strikingly asymmetric)  RA, Mooren’s,Yes Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally,Do Terrien ptsthen need spreads to wear circumferentially protective eyewear? Yes Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 189 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon Does Terrien’s render the cornea significantly thinner Does it havethan a gender normal? predilection?  WithWhile respect once Yesthought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral,Is the or bilateral?thinned doesn’t Terrien’s cornea belong, at risk for rupture and with why? Bilateral (althoughmild trauma? involvement can be strikingly asymmetric)  RA, Mooren’s,Yes Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally,Do Terrien ptsthen need spreads to wear circumferentially protective eyewear? Yes Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 190 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon Does Terrien’s render the cornea significantly thinner Does it havethan a gender normal? predilection?  WithWhile respect once Yesthought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral,Is the or bilateral?thinned doesn’t Terrien’s cornea belong, at risk for rupture and with why? Bilateral (althoughmild trauma? involvement can be strikingly asymmetric)  RA, Mooren’s,Yes Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally,Do Terrien ptsthen need spreads to wear circumferentially protective eyewear? Yes Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 191 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon Does Terrien’s render the cornea significantly thinner Does it havethan a gender normal? predilection?  WithWhile respect once Yesthought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral,Is the or bilateral?thinned doesn’t Terrien’s cornea belong, at risk for rupture and with why? Bilateral (althoughmild trauma? involvement can be strikingly asymmetric)  RA, Mooren’s,Yes Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally,Do Terrien ptsthen need spreads to wear circumferentially protective eyewear? Yes Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 192 Q Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon Does Terrien’s render the cornea significantly thinner Does it havethan a gender normal? predilection?  WithWhile respect once Yesthought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral,Is the or bilateral?thinned doesn’t Terrien’s cornea belong, at risk for rupture and with why? Bilateral (althoughmild trauma? involvement can be strikingly asymmetric)  RA, Mooren’s,Yes Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally,Do Terrien ptsthen need spreads to wear circumferentially protective eyewear? Yes Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 193 A Concerning PUK Speaking of Terrien’s… Is it a common, or an uncommon condition? Uncommon Does Terrien’s render the cornea significantly thinner Does it havethan a gender normal? predilection?  WithWhile respect once Yesthought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of theIs followingit unilateral,Is the or bilateral?thinned doesn’t Terrien’s cornea belong, at risk for rupture and with why? Bilateral (althoughmild trauma? involvement can be strikingly asymmetric)  RA, Mooren’s,Yes Behçet, IBD Which sector of the cornea is involved first, and how does it progress from there? It starts superonasally,Do Terrien ptsthen need spreads to wear circumferentially protective eyewear? Yes Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 194 Q Concerning PUK Speaking of Terrien’s? Is it a common, or an uncommon condition? Uncommon Does Terrien’s render the cornea significantly thinner Does it havethan a gender normal? predilection?  WithWhile respect once Yesthought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of There is a lookalike condition--rarer than Terrien’s--which differs in that the1)Is following itit unilateral,is moreIs the likelyor bilateral?thinned to doesn’t occur Terrien’s in childr cornea belong,en, at and risk 2)for itrupture is andinflammatory with why? in nature. mild trauma? TheBilateral Cornea (althoughbook involvement speculates can that be itstrikingly might not asymmetric) be a separate condition,  RA, Mooren’s,Yes Behçet, IBD butWhich rather sector a ofmanifestation the cornea is involvedof the same first, anddz processhow does as it progress Terrien. from What there? is it? Fuchs’It starts superonasally,superficialDo Terrien marginal ptsthen need spreads to keratitiswear circumferentially protective eyewear? Yes Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 195 A Concerning PUK Speaking of Terrien’s? Is it a common, or an uncommon condition? Uncommon Does Terrien’s render the cornea significantly thinner Does it havethan a gender normal? predilection?  WithWhile respect once Yesthought to tobe more manifesting common in males, it is PUK, now consid eredwhich equal of There is a lookalike condition--rarer than Terrien’s--which differs in that the1)Is following itit unilateral,is moreIs the likelyor bilateral?thinned to doesn’t occur Terrien’s in childr cornea belong,en, at and risk 2)for itrupture is andinflammatory with why? in nature. mild trauma? TheBilateral Cornea (althoughbook involvement speculates can that be itstrikingly might not asymmetric) be a separate condition,  RA, Mooren’s,Yes Behçet, IBD butWhich rather sector a ofmanifestation the cornea is involvedof the same first, anddz processhow does as it progress Terrien. from What there? is it? Fuchs’It starts superonasally,superficialDo Terrien marginal ptsthen need spreads to keratitiswear circumferentially protective eyewear? Yes Does it affect vision? If so, how?  AndYes, in by inducingthis highgroup? astigmatism  Mooren’s, Terrien’s marginal, Sarcoid, SLE

Why is Terrien’s the oddball in this group? Two reasons: --PUK in the others is an inflammatory process; Terrien’s is noninflammatory --As implied by the word ‘ulcerative’ in the name, the corneal epithelium is disrupted in PUK. In contrast, the epithelium is intact in Terrien’s. 196 Concerning PUK

Fuchs’ superficial marginal keratitis 197 Q Concerning PUK

 All of the following are true concerning Mooren’s ulcer except (could be more than one):  Cause is unknown  One clinical type presents as a unilateral PUK in the elderly  The other type presents as bilateral disease in young African women  Patients with the ‘African’ variety often have a history of systemic helminth infection  Mooren’s responds readily to aggressive local therapy 198 A Concerning PUK

 All of the following are true concerning Mooren’s ulcer except (could be more than one):  Cause is unknown T  One clinical type presents as a unilateral PUK in the elderly T men  The other type presents as bilateral disease in young African women  Patients with the ‘African’ variety often have a history of systemic helminth infection T poorly  Mooren’s responds readily to aggressive local therapy ^ 199 Concerning PUK

 All of the following are true concerning Mooren’s ulcer except (could be more than one):  Cause is unknown T  One clinical type presents as a unilateral PUK in the elderly T men  The other type presents as bilateral disease in young African women  Patients with the ‘African’ variety often have a history of systemic helminth infection T poorly  Mooren’s responds readily to aggressive local therapy ^

Mooren’s ulcer is a chronic, progressive PUK. By definition, the cause is unknown. It starts sectorally, progresses circumferentially, then finally centrally. The leading edge is undermined and de-epithelialized. Two clinical varieties are recognized: Unilateral disease in the elderly, and rapidly progressive, severe bilateral disease that strikes young African men. These men usually are seropositive for helminthic disease.

The plethora of treatments stands as gloomy testimony to the relative ineffectiveness of each. Ocular modalities include topical steroids, BCL, n-acetylcysteine drops, topical cyclosporine and conjunctival resection. Quite often, systemic immunosuppressives are needed: steroids, MTX, and/or cyclophosphamide.