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Overview of Episcleritis vs Episcleritis and Conjunctivitis is common. Episcleritis and Scleritis are not. Thomas F. Freddo, O.D., Ph.D. Apply topical anesthetic. Then use a cotton PPfrofessor an d Former DitDirector swabbt to move th e conjjtilunctival surffhilace while School of Optometry observing through slitslit--lamp.lamp. If the dilated and congested vessels move with the swab, its University of Waterloo conjunctivitis. Advice on holding lid open -roll- roll--upup awning

Episcleritis DDX Sectorial Redness

 A relatively benign, Staph marginal recurrent rarely associated with ulcer systemic disease. Limbal  Visual acuity is normal phlyc tenu le  Mild, occasional pain Limbal  More often in women than men, as is malignancy generally true for most  autoimmuneautoimmune--typetype  Two varieties: simple & Simple Episcleritis nodular

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EPISCLERITIS Simple Episcleritis

 Often recur at 1- to 3-month intervals.  Episodes appear acutely  Most cases are idiopathic occurring more often and usually last 7-10 days in women than men and usually in their 30’s or  Most resolve after 2-3 40’s. weeks.  Prolonged episodes may be  A preliminary workwork--upup is worth considering only more common in patients if your directed case history leads to suspicion with associated systemic of an underlying systemic association. conditions.  Systemic associations found primarily through  Some patients note that history in 1/3 of patients. Labs are to confirm. episodes are more common  No correlation between type, laterality or in the spring or fall. recurrences and presence of associated  Precipitating factor is rarely systemic disease found.

EPISCLERITIS Nodular Episcleritis

Takes longer  Virtually any infectious disease can be to resolve (4-6 associated with episcleritis – CBC with differential , looking for leukocytosis/leukocytosis/eosinophiliaeosinophilia weeks)  Bacteria, including , , and  Viruses, including herpes  Fungi  Parasites  Other more common causes –  - rule out by history and skin appearance  Foreign Body – rule out by history and exam Normal = 7.1  Chemicals – rule out by history, measuring tear pH and exam   Adult  Seronegative spondyloparthropathies

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If the patient is unresponsive to treatment a referral EPISCLERITIS to an /external disease specialist is prudent in order to explore the more rare causes including: Differential Diagnosis

 Other rare causes/associations  Systemic erythematosus  Gout  TT--cellcell leukemia  Paraproteinemia Adult Rheumatoid arthritis  Paraneoplastic syndromes - Sweet syndrome, dermatomyositis  WiskottWiskott--AldrichAldrich syndrome Seronegative spondyloarthropathies  Adrenal cortical insufficiency  Necrobiotic xanthogranuloma  Progressive hemifacial atrophy  Following transscleral fixation of posterior chamber intraocular  Insect bite granuloma  Malpositioned Jones tube

EPISCLERITIS Differential Diagnosis

Differential Diagnosis AUTOIMMUNE DISEASES  GoutGout–– acute attacks of foot or lower joint pain especially in great toe but any synovial joint can be involved. Elevated uric acid level as a result of a  Adult rheumatoid arthritis defect in purine metabolism. Results in deposition of crystallized uric acid as “tophi”. R/O in history or exam . Get urine sample for uric acid level . In acute attacks white count elevated with left shift and sed rate (ESR) may be  Seronegative spondyloarthropathies elevated. Diet. MedicationMedication of choice allopurinolallopurinol..   Inflammatory bowel disease   Reactive arthritis (Reiter’s syndrome)

Removal of tophi

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If the patient is unresponsive to treatment a referral to an Episcleritis/Scleritis and relationship with the cornea/external disease specialist is prudent in order to seronegative spondyloarthropathies explore the more rare causes including:

 Read and review:  Other rare causes/associations  Systemic Lupus erythematosus  Polyarteritis nodosa www.arc.org.uk/about_arth/med_report  TT--cellcell leukemia s/series4/tr/6604/6604.htm  Paraproteinemia  Paraneoplastic syndromes - Sweet syndrome, dermatomyositis  WiskottWiskott--AldrichAldrich syndrome  Adrenal cortical insufficiency www.orthop.washington.edu/uw/tabID_  Necrobiotic xanthogranuloma  Progressive hemifacial atrophy 3376/print_full/ItemID_130/mid_0/Arti  Following transscleral fixation of posterior chamber intraocular lens cles/Default.aspx  Insect bite granuloma  Malpositioned Jones tube

Episcleritis – Work-up Treatment

 Ocular therapy Useful laboratory studies in this  Simple episcleritis often requires no treatment. Cold compresses and iced artificial tears are useful for patients with mild-to-moderate symptoms. Patients group of patients include: with severe or prolonged episodes may require topical but  serum uric acid - Gout steroids have been shown to worsen recurrences.  complete blood count with  Nodular episcleritis is more indolent and may require local drops or anti-inflammatory agents. differential  Topical pred phosphate penetrates the eye less well and thus may be preferred  rheumatoid factor over acetate in these cases to mitigate against steroid response.  erythrocyte sedimentation rate  Systemic therapy  If nodular episcleritis is unresponsive to topical therapy, systemic anti-  Venereal Disease Research inflammatory agents may be useful. Laboratory (VDRL) test  Flurbiprofen (100 mg tid) is usually effective until is suppressed.  fluorescent treponemal antibody  If there is no response to flurbiprofen, indomethacin should be used; 100 mg absorption (FTA-ABS) daily and decreased to 75 mg when there is a response.  chest x-ray  Many patients who do not respond to one nonsteroidal anti-inflammatory agent (NSAID) may respond to another NSAID.  Activity  Sunglasses may be useful for patients with sensitivity to light.

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Episcleritis vs. Scleritis Pain in the Differential

 View patient in  MOST Important - judge DDx: Severe ocular daylight and/or red- the pain. If it is deep pain: free. penetrating pain, Corneal surface radiating all around the  Episcleritis will look problems red, scleritis will head and/or awakens the Angle closure have a deeper, patient from sleep, think purple-red scleritis. OTC coloration not well will not lessen pain. Scleritis, especially seen by slit-lamp.  If pain worsens with eye with anterior . movement, think scleritis.

Note deep purple-red background

EPISCLERITIS VS SCLERITIS: Use 2.5% phenylephrinephenylephrine.. Generally, it will whiten an episcleritisepiscleritis,, not a deep scleritis Scleritis

Most common 4th4th--6th6th decade Episcleritis In 15% of cases, scleritis is the presenting sign of collagen vascular disease. More in females Scleritis 1.6:1 OneOne--thirdthird are recurrent

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Scleritis Anterior Scleritis

 Rheumatoid arthritis is the underlying disease for approximately one sixth of patients suffering from scleritis  Anterior  Other connective tissue and autoimmune diseases seen with Diffuse - most scleritis include the following: common  Systemic lupus erythematosus(SLE) Nodular  Polyarteritis nodosa Necrotizing  Seronegative spondyloarthropathies Ankylosing spondylitis Most cases stay as Psoriatic arthritis same type as at Reactive arthritis original Inflammatory bowel disease presentation, but  Sarcoidosis some advance  Tuberculosis  Syphilis  Lyme *List is almost identical to anterior uveitis

Treatable Masquerades Diffuse Anterior Scleritis

 Most common form Infectious scleritis is a possibility that should be  Insidious onset over 5-10 evaluated. days Herpes scleritis can present as a masquerade of  Easily misdiagnosed as scleritis and should always be considered if the episcleritis patient has unilateral scleritis.  Increased scleral Syphilis is another important masquerader that translucency when needs to be ruled out, as it is treatable. (Luetic healed serology)  Least associated with Conditions such as severe or severe systemic disease atopy or allergy can also induce scleritis, so a thorough patient history is essential.

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Necrotizing Anterior Nodular Scleritis Scleritis

Nodule firm May occur and immobile with or Onset insidious without iflinflamma tion

Necrotizing Scleritis Wegener’s Granulomatosis

INFLAMMATORY NON-INFLAMMATORY Usually begins with respiratory symptoms  Runny nose, with puspus--filledfilled discharge Nosebleeds  Sinus pain and inflammation (sinusitis)  Most severe and  Scleromalacia perforans  Hoarse voice destructive form  Painless melting of  29% di e withi n 5 yrs of  Inflammation of the gums leaving exposed  Ear infections onset to  50-90% have underlying  Cough disease, usually  Spontaneous perforation  Chest pain rheumatoid arthritis, rare  Coughing up blood ((hemoptysishemoptysis)) Wegener’s, relapsing  Most often with  Shortness of breath polychondritis rheumatoid arthritis  Wheezing  General ill feeling (malaise)  But can present with scleritisor granulomatousanterior uveitis

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Work up of Wegener’s ANCA

 p-ANCA, or protoplasmic-  AntiAnti--neutrophilneutrophilcytoplasmic antibodiantibodieses (ANCAs) are a staining antineutrophil group of autoantibodiesautoantibodies,, mainly of the IgGtype, against cytoplasmic antibodies, antigens in the cytoplasmcytoplasm of neutrophilsand monocytes.monocytes. show a perinuclear  They are detected in a number of conditions associated staining pattern. with systemic vasculitisvasculitis,, so called ANCEANCE--associatedassociated  c-ANCA, or classical vasculiditiesvasculidities,, including Wegener’s granulomatosis; antineutrophil cytoplasmic microscopic polyangitis and Churg–Strauss syndrome. antibodies, show a  ANCA were originally shown to divide into two main diffusely granular, classes, cc--ANCAANCA and pp--ANCA,ANCA, based on the pattern of cytoplasmic staining staining on ethanol-ethanol-fixedfixed neutrophils and the main target pattern. antigen. ANCA titers can also be measured using ELISA  It is cc--ANCAANCA that is primarily and indirect immunofluorescence.immunofluorescence. associated with Wegener’s

Posterior Scleritis Summary of Work-up

Vascular congestion with hemorrhage, reduced  Patients with scleritis need to be evaluated for potential systemic vision with macular stria – but with PAIN! diseases. I would recommend that the scleritis work-up be done by a rheumatologist due to the wide range of conditions.  Laboratory studies are likely to include both serologic tests and urinalysis.  Luetic serology – FTA-Abs and VDRL/RPR  CBC with diff  Anti-neutrophil cytoplasmic antibody (ANCA) studies will test for Wegener’s granulomatosis.  Various vasculidities can affect kidney and so urine protein should be measured.  R/o TB with CXR and PPD

B-scan: Flattening of posterior pole, Choroidal and scleral thickening with sub-Tenon’s fluid accumulation and “T-sign”

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Based on the past history, review of systems, and , select appropriate diagnostic tests to confirm or reject the following suspected associated diseases: Treatment of Scleritis

 Rheumatoid factor - Rheumatoid arthritis Oral NSAIDS first line, either COX-1 or more  Antinuclear antibodies - Systemic lupus erythematosus,erythematosus, rheumatoid arthritis, polymyositispolymyositis,, progressive systemic sclerosis, or mixed connective tissue recent COX-2 such as Celebrex at 100-200 mg  Antineutrophil cytoplasmic antibodies (ANCA) - Wegener granulomatosisgranulomatosis,, polyarteritis nodosanodosa,, or microscopic polyangiitis qd. More difficult cases may require oral  Human leukocyte antigen (HLA) typing - Ankylosing spondylitis, reactive arthritis , steroids or immunosuppressants. If NSAID use psoriatic arthritis, or arthritis associated with inflammatory bowel disease  Eosinophil count/immunoglobulin E ((IgEIgE)) - Allergic angiitis of Churg-Churg-StraussStrauss chronic, protection of GI system may be syndrome or atopy required (omeprazole-Prilosec)  Uric acid -Gout  Erythrocyte sedimentation rate (ESR) - Giant cell arteritis Treatment should be coordinated with  Hepatitis B surface antigen ((HBsAgHBsAg)) -Polyarteritis- Polyarteritis nodosa  Serologies - Infectious diseases, including syphilis and Lyme disease rheumatologist of an ophtho with specialty  PurifiedPurified--proteinprotein derivative (PPD) skin test - Tuberculosis training in ocular immune disease.  Anergy skin test -Sarcoidosis  Prick test -Atopy- Atopy

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