Etiologies 85 causes 50% idiopathic conditions 20% trauma 20% systemic 10% local (H Zoster, Toxoplasmosis, etc)

1 Doc I have eyeritis Uveitis front to back Brian E. Mathie. OD, FAAO 2 Demographics Most commonly in patients 20-59 years old 5-10% in pts<16yo Males = Females All races affected equally Ocular history prior episodes and their treatment; surgery; trauma 3 Uveitis 3 rd most common preventable cause of blindness in US Anterior uveitis most common form (8:100,000) Anterior uveitis etiology known in 50% of cases 4 Etiologies 85 causes 50% idiopathic conditions 20% trauma 20% systemic 10% local (H Zoster, Toxoplasmosis, etc) 5 6 7 Etiologies Idiopathic Implies that all other potential causes have been evaluated and appropriately ruled out Symptoms Redness Aching pain 1

Aching pain Photophobia Tearing Pain with accommodative tasks Browache download immunologic history form at http://www.uveitis.org/ Enhanced/Review/ques.htm 8 9 Clinical Findings Circumlimbal injection, not always present Eyelids may be involved Corneal precipitates and edema Fixed and/or irregular pupil Varying IOP initial decrease due to inflammation of ciliary body possible future increase due to decreased outflow no change in pressure Cells and/or flare, rarely a hypopion Band keratopathy, cataract Posterior synechia Retina-r/o detachment, cystoid macular edema (CME) Summed Ocular Inflammation Score (SOIS) Anterior chamber cell grading: 0 for 0 cells, 0.5 for 1-5(trace) 1 for 6-15, 2 for 16-25, 3 for 26-50, and 4 for >50 10 Masquerade syndromes Pigment dispersion syndrome 2

Pigment dispersion syndrome Pseudoexfoliation Acute angle closure also has corneal edema, IOP, pupil irregularity Intraocular melanoma Intraocular/Orbital lymphoma Corneal ulcers Retinal detachment AC cells imply subacute or chronic timeframe 11 Classification Timing Acute vs Chronic or insidious Course Limited (<3 months) Persistent(>3 months) 12 13 14 Timing Acute Sudden onset and limited duration Recurrent Repeated events > 3 months between occurrence Chronic Persistent Relapses after < 3 months off treatment Classification Anatomical Location Anterior (Anterior Chamber) Usually idiopathic or HLA B27 (-) Intermediate (Ciliary Body) Posterior (Retina and Choroid) Panuveitis (All structures) New Treatment The FDA has granted AbbVie s adalimumab (Humira) orphan drug designation for the treatment of non-infectious intermediate, posterior, or pan-uveitis, or chronic non-infectious anterior uveitis. 3

designation for the treatment of non-infectious intermediate, posterior, or pan-uveitis, or chronic non-infectious anterior uveitis. AbbVie is investigating the efficacy and safety of the drug for the treatment of non-infectious uveitis, which is currently in phase III development. The drug is not currently approved to treat any form of uveitis. MAY 27, 2014 15 16 Anatomical Classification Based on where the primary cause is located- edema of optic nerve or CME doesn t count Classification Granulomatous Mutton Fat keratic precipitates Often involves systemic or autoimmune conditions, syphilis, Lyme disease, TB, toxo, sarcoid, herpetic Nodules-Koeppe, Bussaca and Berlin Nongranulomatous Anterior chamber Small cells, small KP Idiopathic HLA B27 sarcoid, herpetic, Fuchs 17 IOP/ Iris Clues Decreased in acute phase Increased in herpetic etiologies o HSV-diffuse iris atrophy o HZO-sectoral iris atrophy o Fuch s-pigment loss on iris o Rubella Virus? 18 Exam Look posterior to cells 4

Look posterior to cells Lens-cataract Vitreous-cells, debris, haze Retina-infiltrates, necrosis, retinitis, detachment Choroid-infiltrates, scarring Optic Nerve-edema is common 19 20 Treatment Topical Steroid 1% prednisolone acetate is the gold standard Q15min to Q1h Need night time coverage (gel, ung) Soft steroids only ones approved for iritis but not 1 st choice Taper 2-4 weeks, at least as long as presentation Durezol: half dosing vs prednisolone acetate Lotemax: for very long tapering Treatment Based on classification of uveitis Anterior Uveitis=Iritis=Anterior Cyclitis-idiopathic Intermediate-peripheral retina, pars plana and vitreous, only 4-8% of uveitis cases 69% idiopathic, 22% Sarcoid Posterior- highest risk of vision loss 21 NSAIDs Topical NSAID May help with pain, reduce CME Nevenac (nepafenac0.1%) TID Xibrom (bromfenac) BID Bromday (bromfenac) QD 22 Cycloplegics Cycloplegics 5

Cycloplegics 5% homatropine BID or TID 0.25% scopolamine BID 1% atropine avoid in most all cases to avoid synechia 23 Glaucoma medications Alphagan/alpha agonists favored CAI s only fair; Beta blockers often contraindicated Avoid prostaglandins and pilocarpine 24 Treatment Uveitic Glaucoma 0.5% timolol BID (Betimol, Istalol, Timoptic (XE)) 0.1 % brimonidine TID (Alphagan) Contraindicated Treatment Pilocarpine Prostaglandin Analogs 25 26 NSAIDs Topical NSAID may help in pain management May also reduce CME Steroid injections Subtenon Triamcinolone.5cc Kenalog repository Side Effects infection cataract (17.5%) ocular hypertension (36%) hemorrhage retinal detachment Intraocular Steroid Injection 6

Intraocular Steroid Injection 27 28 29 30 Orals Orals ibuprofen 600-800mg t.i.d. acutely; consider Celebrex for prophylaxis in chronic or recurrent cases; Refer for oral steroids IF systemic ~ 60 mg/day baseline dosage If Advanced Immunosuppressives Methotrexate, cyclosporine et al. Injectable cytokine blockers Remicade, Enbrel, and Humira (newer) use is increasinging from systemic to ocular arena in sight threatening cases. Zenapax (newest) When to order testing Recurrent Bilateral presentation Positive findings with review of systems Granulomatous findings Involvement of the posterior aspect Severe Lab testing for systemic disease CBC - Complete Blood Count CRP - C Reactive Protein ESR - Erythrocyte Sedimentation Rate HLA B-27 - Human Leukocyte Antigen ANA - Anti-Nuclear Antibody (Lupus) RPR - Rapid Plasma Reagin (Syphilis) FTA- ABS - Fluorescent treponemal antibody absorbtion test (Syphilis) ACE - Angotenesin converting enzyme (Tuberculosis) PPD - Purified protein derivative (Tuberculosis) RF - Rheumatoid factor (Rheumatoid Arthritis) 7

31 32 Etiologies idiopathic ocular trauma ocular surgery systemic inflammatory disease Systemic disease Clues repeating cases abnormally aggressive unresponsive to treatment bilateral alternating unilateral recurrences 33 Systemic Diseases HLA B27 ankylosing spondylitis 80% male Sacroilitis bamboo sign on spine Upper lung fibrosis Reiter s syndrome (Reactive Hans Conrad Reiter) Cant see, cant pee, cant climb a tree psoriatic arthritis 34 HLA-B27 Behcet s oral and genital ulcers 81% Asians, 13% Caucasians 86% develop eye disease (in Japan) Findings-hypopion, vitritis, ION, retinal vasculitis Diagnosis criteria Recurrent oral ulcers (3x in 1 yr) and 2 of» Ocular inflamation» Skin lesions 8

» Skin lesions» Recurrent genital warts» Pathergy test 35 Systemic Diseases Sarcoidosis Multiple Sclerosis (MS) Syphilis Lyme Disease Histoplasmosis Rheumatoid Arthritis Juvenile Rheumatoid Arthritis 36 Rheumatoid Arthritis 75% female, esp. Anglo-Saxons 1-3% of Americans JRA now called JIA (juvenile idiopathic arthritis), follow children more closely if younger than age 7, +ANA, HLA-DR5 + antigen-antibody reaction of rheumatoid factor against IgG triggers release of cytokine TNF-alpha joint inflammation of synovial membrane and cartilage 37 Rheumatoid Arthritis Check for rheumatoid factor=antibody to IgG; HLA-DR4 and/or HLADR5 surface antigens present in 80% of all RA patients X-rays Treatment options NSAIDS, DMARDS (disease-modifying antirheumatologic agents) such as steroids, plaquenil, gold, sulfasalazine, and Remicade 38 Herpes Simplex Number one cause of infectious uveitis 85% unilateral 9

85% unilateral Disciform keratitis presents extra risk for uveitis..watch IOP! HSV s big three: unexplained corneal scarring corneal desensitivity iris atrophy Oral treatment of herpes simplex uveitis: 400 mg acyclovir 5x dailyfor 7-10 days 500 mg valacyclovir t.i.d. for 7-10 days 250 mg famcyclovir t.i.d. for 7-10 days. 39 Herpes Simplex Herpetic Eye Disease Study (HEDS) prophylactic antiviral dosing, esp. valacyclovir 500mg qd to bid for > 1 year in keratitis and keratouveitis. Advocate oral treatment! Polymerase chain reaction (PCR) to amplify and identify viral DNA from small specimens, e.g. aqueous humor Other keratitis, blepharoconjunctivitis, trabeculitis, scleritis 40 41 42 Herpes Zoster vesicular eruption along V1 by varicella virus systemic treatment Zovirax and prodrugs Valtrex and Famvir topical antivirals ineffective, steroids are therapeutic cornerstone monitor closely, treat aggressively uveitis may be rapid onset, severe extra vigilance if corneal findings of spk/mucoid plaques Inflammatory bowel disease Crohn s disease, ulcerative colitis 2 million Americans, >50% female chronic intestinal inflammation mediated by TNF-alpha UC less likely to cause uveitis than Crohn s 3-10% have ocular involvement, mainly episcleritis and uveitis granulomatous uveitis may be bilateral, posterior, and chronic 50% risk for uveitis if arthritic! Seronegative spondyloarthropathies 10

~ 350,000 Americans RF negative, but HLA-B27 positive Ankylosing spondylitis, reactive arthritis (formerly Reiter s), psoriatic arthritis, and undifferentiated form Morning back pain, improved with exercise Usually acute, unilateral uveitis most common cause of hypopyon uveitis most common cause of uveitis (30%) that is confirmed 43 Sarcoidosis A series of inflammatory nodules, mostly lung but also eyes, joints, skin, liver, lymphatics, spleen, and kidney; unknown origin More prevelant in African-Americans and European whites Often dx d by chest x-ray and physical exam (lymphadenopathy, fever, respiratory problems) angiotensin converting enzyme (ACE), serum calcium + lysosyme, and biopsy (characteristic coffin-shaped inflammatory cell). +ACE may specify sarcoid s presence but ACE does not rule it out Classically bilateral Frequently involves posterior segment 44 Sarcoidosis Tx: steroids! 20% show ocular involvement: uveitis tends to be chronic, unilateral, granulomatous, and more likely anterior/intermediate Beware of EOM dysfunction, optic neuropathy, and retinal vasculitis (candlewax drippings) 45 Fluoroquinolones Association considered probable Mean time to onset of anterior uveitis is 13 days Resolves with fluoroquinolone discontinuation 46 11

47 48 49 Case 1 29 YOWM C/O pain OD x 1 day after being hit by wire, blurry vision and photophobia SLE-injection 1+ diffuse, SPK OD, cells 1+ OD Traumatic Iritis May have photophobia in other eye Immediate tx: cycloplegic Testing: IOP, measure cell and flare, retinal evaluation Prescribe: steroid if severe, cycloplegic, sunglasses 50 51 52 Longstanding Iritis Post Cataract Surgical Iritis Anterior Uveitis Day 1 Gr rare to Gr 1 Day 7 Gr rare to trace Day 28 0 Glaucoma and Uveitis TM blocked by inflammatory cells, debris TM inflamed (Posner Schlossman Syndrome) Anterior synechiae Posterior synechiae CB inflamed Steroid induced 53 Other consequences of uveitis Retinal damage Macular Edema 12

Macular Edema Cataract 54 55 56 57 Case presentation June 15, 2010 Visit #1 18-year-old Caucasian female red and swollen right eye for 3 days throbbing pain, tearing, light sensitivity POHx: contact lens wear OD only PMHx: ovarian infection treated with cephalexin 500 mg PO TID FMHx: (+)hypertension and diabetes SHx: (+)smoking and alcohol consumption Allergies: NKDA Young Iritis 18YOWF Visit #1 VA: 20/70-1 OD, 20/20 OS SLE: I + - II conjunctival and episcleral injection OD II + cells, I + flare OD cornea clear OD/OS unremarkable OS Young Iritis June 15, 2010 Visit #1 Assessment: Anterior Uveitis OD Plan: Pred Forte Q 1HR OD Xibrom BID OD Follow up 1 week with DFE Not So Obvious 22 YOWM Mentally retarded, noncommunicative Reduced vision and eye rubbing noticed by care givers Clinical Findings Conjunctival injection GII+ circumlimbal Cells G III 13

Cells G III Flare G II+ Ta 28 OD and 19 OS 58 59 60 Treatment Pred Forte OD Q1 hr Homatropine OD BID F/U in 2 days-slightly better F/U in 1 week- much better, taper over 3 weeks F/U 3 weeks-full blown iritis return, APPROPRIATE Diagonosis Retinal detachment OD with a high myope Anterior and posterior uveitis secondary to retinal detachment Refer to retinologist for RD repair When to work up Bilateral Recurrent Granulomatous Severe Posterior Synechiae + Systemic involvement + Physical exam findings 61 Thank You Brian Mathie, OD, FAAO bmathieod@aol.com 14