boards’ fodder Vasculitides Anna Chacon, MD Diagnosis Epidemiology Pathogenesis Clinical Diagnostic Pathology Therapy Complications/ Features Approach Associations SMALL VESSEL VASCULITIS Cutaneous small vessel vasculitis [(CSVV), cutaneous leukocytoclastic vasculitis (LCV)/angiitis, hypersensitivity angiitis, allergic vasculitis, cutaneous necrotizing venulitis] Henoch- Most common IgA-dominant immune 1 - Intermittent Palpable purpura Necrotizing Self-limited, supportive. Renal vasculitis Schönlein purpura in children < 10 deposits in small palpable purpura on (required) w/ vasculitis. Dapsone & colchicine often mild but can (anaphylactoid yrs; associated w/ blood vessel walls. extensor extremities at least 1 of the Immunoreactants may decrease duration be chronic. May purpura, purpura preceding respiratory Dx supported by IgA & buttocks following: arthritis/ deposited in of skin lesions. Systemic be associated rheumatica, infection- 75% by predominance in the 2 – arthralgias, arthralgia, diffuse skin are IgA. steroids: Rx for arthritis w/underlying CSVV secondary GAS. May be seen correct clinical setting. arthritis abdominal pain, renal DIF: perivascular & abdominal pain. malignancy in to circulating in adults. Slight male 3 - abdominal pain/ involvement, bx w/IgA IgA, C3 & fibrin Nephrology referral w/ adults. IgA immune predominance. melena; bowel deposition. deposits. renal involvement. complexes) angina/ischemia 4 – renal involvement (hematuria & RBC casts). Acute Seen primarily in Unknown. 75%: Benign clinical Routine labs: LCV involving Spontaneous resolution Extracutaneous hemorrhagic children 4-24 months associated w/infxn, course. Annular, nonspecific. Dx based capillaries & within 1-3 weeks. involvement rare. edema of infancy of age. Child is well drug exposure, circular, or targetoid on clinicopathologic postcapillary Antihistamines for Two-thirds of pts (Finkelstein’s appearing. or immunization. purpuric plaques on correlation. venules of upper symptomatic relief. have infectious dz, Seidlmayer Thought to involve face & extremities. Characteristic: age & mid-dermis. DIF: prodrome. syndrome, immune complex Tender, non-pitting onset < 2 yrs., IgA deposits in purpura en deposition edema of acral disease confined to vascular pattern in cocarde responding to sites. skin, brief duration. 1/3 – 1/4 of cases. avec edema, antigenic trigger. postinfectious cockade purpura) Urticarial Uncommon. Peak Complement Painful/burning Lab: ESR, CRP, SPEP, Typically hive Difficult. Try: Many pts have vasculitis (chronic incidence: 5th decade. activation triggers urticarial lesions ANA, autoantibodies w/edema but antihistamines, dapsone, hives persisting urticaria as a 60-80% female. mast cell release lasting >24 hrs; (anti-SS-A/Ro, also w/LCV. A NSAIDS, antimalarials. >24 hrs, but few manifestation of Hypocomplementemic of inflammatory resolve leaving anti-SS-B/La), perivascular Unresponsive/ have urticarial venulitis, urticaria form: almost mediators. Idiopathic behind hemosiderin, complement levels lymphocytic systemic: steroids & vasculitis w/signs & arthralgia w/ exclusively in women. or associated with causing red- (CH50, C3, C4). infiltrate is not perhaps additional & sxs. Systemic: necrotizing SLE, Sjӧgren’s, brown maculae/ Further evaluation can enough for dx. immunosuppressants. arthritis, nephritis, angiitis) cryoglobulinemia, postinflammatory include: CXR, PFTs, abdominal or Wegener’s hyperpigmentation. RFTs. distress. Lung granulomatosis. involvement can be fatal. Erythema Uncommon, rare. Suspicion that Symmetrical, slowly Skin biopsy. LCV w/thickening Limited: intralesional Association with elevatum diutinum Can develop at any streptococci are developing red- Serum protein of vessel walls; or high-potency topical IgA monoclonal (extracellular age. No racial/gender trigger for chronic brown papules & electrophoresis later – fibrosis, steroids. Widespread/ gammopathy cholesterosis) predilection. immune complex nodules favoring (SPEP). granulomatous resistant: dapsone. or even multiple reaction. Occurs backs of hands, inflammation, Nicotinamide & myeloma. Earlier in HIV/AIDS, over digital joints, occasional tetracycline may help. onset more often unclear if result of knees, & elbows. cholesterol in HIV setting. other factors vs. Rarely more deposits. immunosuppression. widespread. Usually asymptomatic. Secondary causes of CSSV: Drugs; infections; malignancies, most often hematologic SMALL & MEDIUM-SIZED VESSEL VASCULITIS (“MIXED”) Cryoglobulinemia Frequency varies Circulating Ig Type I: thrombosis, Blood must be Papular lesions Avoid cold. Treat Peripheral [types I, II, III; w/geography, may complexes that livedo, Raynaud transported to lab show LCV, while underlying disease. neuropathy & types II & III: reflect prevalence precipitate when syndrome, ulcers. at 37°C then cooled necrotic or If HCV involvement: glomerulonephritis “mixed”); “Types 2 differences in HCV. incubated at <4° C. Types II-III: chronic to < 4° C; cryocrit ulcerated lesions interferon + ribavirin. can develop. & 3, LCV” Higher prevalence in Compositions: I – immune complex is then determined may demonstrate Progressive: MTX or Types II-III: most Southern Europe. monoclonal IgG or vasculitis, skin & & protein selectively medium-sized cyclophosphamide + commonly in the IgM; II – monoclonal kidneys. analyzed. Reactive vessel vasculitis. systemic steroids, +/- setting of HCV IgM + polyclonal IgG; hypocomplementemia. DIF: granular plasma exchange. infection, 80%. III – polyclonal IgG. deposits w/IgM & C3 in vascular pattern in papillary dermis. Microscopic Estimated incidence Unknown. May Palpable purpura, Presence of P-ANCA. Segmental 2 phases: More severe polyangiitis 3-24/million. Men > be associated erythematous Additional testing: necrotizing 1) Remission – renal vasculitis (microscopic women. Mean age w/infectious macules & CXR, EMG/nerve vasculitis of initially steroids, favors older pts. polyarteritis, of onset = 57 years, endocarditis. patches, splinter conduction studies, smallest blood cyclophosphamide or ANCA persistence microscopic peak 65-75 yrs. Medications/ hemorrhages & lung/nerve/kidney bx. vessels & less rituximab for significant despite remission polyarteritis malignancy may be ulcers. Constitutional often of small/ organ involvement. – increased risk of nodosa) a trigger. ANCA is sxs, crescentic medium-sized 2) Maintenance: MTX, relapse. thought to play a role. necrotizing arteries. Absence azathioprine, MMF, IVIg. glomerulonephritis of granuloma Plasma exchange may be & alveolar formation. beneficial. Anna Chacon, MD, is hemorrhage. Wegener’s Incidence = Unknown. Infections, Stages - 1st: general Tissue dx: usually Triad: necrotizing Fauci regimen: Frequency of a preliminary resident granulomatosis 0.3/100,000 annually; including S. aureus, signs & sxs – fever, airway/renal bx, LCV, necrosis, prednisone & organ involvement: (granulomatosis USA: prevalence are suspected malaise, upper sometimes skin helps. granuloma cyclophosphamide. lungs 95%, upper at Orlando Regional with polyangiitis) 1/25,000. Most triggers. airway problems. Investigate: upper formation. Unresponsive: agents airway 90%, Medical Center. She often in Caucasians. 2nd: lower airway: airways, kidneys, Granulomas can be increased. kidneys 85%, joints Slight female cough, dyspnea, lungs. can be in vessel If + response, 70%, eyes 60%, will be a dermatology predominance. hemoptysis, pleurisy. C-ANCA positive walls or adjacent; taper steroid. Other ears 60%, skin 3rd stage: in 50% during early palisading, or immunosuppressants 45%, nerves 20%, resident at the generalized phase, >90% when rich in giant cells. under investigation: heart 10%. involvement generalized. Irregular necrosis – Cyclophosphamide University of Maryland ANCA-Associated Vasculitides incl. skin. Skin: “geographic.” induction followed Medical Center polymorphic by MTX. Recurrence/ beginning July 2014. picture including localized: co-trimoxazole. LCV, urticarial vasculitis, necrotizing pyodermas, panniculitis. Oral ulcers, recurrent epistaxis, nasal septal perforation irinections D Residency p. 4 • Summer 2014 boards’ fodder Vasculitides (cont.) Anna Chacon, MD Diagnosis Epidemiology Pathogenesis Clinical Diagnostic Pathology Therapy Complications/ Features Approach Associations Double dose SMALL & MEDIUM-SIZED VESSEL VASCULITIS (“MIXED”) of Boards’ Churg-Strauss Extremely rare. Unknown. Asthma > 80%, Tissue dx: skin or Striking Very steroid Multi-organ syndrome 0.3/100,000 yearly, Speculation: role often presenting lung. Investigate palisading responsive – i.e. involvement: Fodder! (allergic perhaps associated of leukotriene symptom. Later: lungs & other granulomas w/ prednisolone. Reserve mononeuritis granulomatosis w/atopy. antagonists, pulmonary organs based on marked necrosis, immunosuppressants multiplex 60%, In addition to & angiitis, vaccines, rapid D/C infiltrates, signs & sxs. both associated for tx failures or life- kidneys 50%, this issue’s granulomatous of corticosteroids, vasculitis. Transient Labs: elevated ESR, w/vessels & at a threatening dz. heart 40%, vasculitis of desensitization may pulmonary hypereosinophilia, distance. Marked Both IFN-a & IVIg have GI tract 40%. Boards’ Fodder, Churg-Strauss) trigger disorder. eosinophilic elevated IgE, eosinophilia, shown promise. Localized Vasculitides, don’t infiltrates occur. cryglobulins, nuclear dust, granulomas: Granulomatous immune complexes. giant cells. sometimes forget to download inflamxn of Both cANCA & limited to the new Board’s myocardium