Uveitis

UVEITIS Uveal • • •

Tract Choroid, ciliary body, iris Anterior uveal tract – slitlamp is best Posterior – direct/indirect/slitlamp+direct lenses

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UVEITIS • 20-50 years of age • 10-15% of legal blindness in developed countries • Developing>developed due to infxn like toxo and TB ClinFind • Anterior uveitis o Most common o Unilateral o Acute onset o Pain, photophobia, blurred vision o Circumcorneal redness with minimal palpebral conjunctival injection or discharge o Miosis o Anterior chamber = iritis o Anterior chamber + anterior vitreous = iridocyclitis o Decreased corneal sensation = HSV or HZ o Inc IOP = HSV, HZ, toxo, syph, sarcoidosis (glaucomatocyclitic crisis = Posner-Schlossman syndrome) o Large clumps of white cells and inflame debris (keratic precip) = mutton fat or granulomatous o Small clumps = nongranulomatous o Stellate clumps o Granulo and nongranulo – usually loc inferiorly at wedge-shaped region, arlt’s triangle o Stellate – entire corneal endoth; HSV, HZ, toxo, Fuch’s heterochromic iridocyclitis, sarcoidosis o Iris nodules  Margin = Koeppe nodules  Within stroma = Busacca nodules  Ant chamb angle = Berlin’s nodules o Hypopyon – layering of inflame cells in the inf angle from severe anterior chamber inflame  HLA-B27 Uveitis – most common cause of hypopyon uveitis in Nam and Eu  Behcet’s disease – Asia

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Sectoral/patchy atrophy or transillumination – HSV, HZ o Diffuse atrophy or transillumination – Fuch’s heterochromic iridocyclitis Intermediate uveitis o Cyclitis, peripheral uveitis, pars planitis o Second most common IO inflam o Hallmark: vitreous inflame o Bilateral o Late teens or early adult o M>F o Floaters and blurred vision (Note: pain, redness and photophob absent or minimal) o Most striking finding on exam – vitritis + vitreous condensates (freefloating – snowballs; layered over pars plana and CB – snowbanking) o Cause is unknown in the majority o Sarcoidosis and MS – 5-10% o Syph and TB – uncommon o Most common complications – CME, retinal vas, neovas of OD Posterior uveitis o Retinitis, choroiditis, retinal vas, papillitis (optic neuritis) o Floaters, loss of visual field or scotomas, dec vision o RETINAL DETACHMENT – infrequent; tractional, rhegmatogenous, or exudative o

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LABS ARE ONLY INDICATED IF: • Recurrent • Severe • Bilateral • Granulomatous • Intermediate • Posterior • Diffuse • Fails to respond to std tx

Jaybz D6 Med Class 2009

***A remote hx of BCG vaccination should not preclude PPD testing, since the test should become negative (<5 mm) within 5 years after vaccination. DiffDx • Conjunctivitis – presence of discharge and redness involving both palpebral and bulbar conj • Keratitis – presence of epith staining or defects or by stromal thickening or infiltrate • Acute angle closure glaucoma – markedly elevated IOP, corneal haziness and edema, and a narrow ant chamb angle (uninv eye) Complications & Sequelae • In Ant uv o Ant and post synechiae o Ant syn  impede aq flow  glaucoma o Post syn  if extensive, secondary angle closure glaucoma by pupillary seclusion and forward bulging of the iris (iris bombe) • In Ant and post uv o Lens thickening and opacification o Early: simple shift in refractive error – myopia  cataract in the end • CME – common cause of visual loss in severe ant and intermediate uv o FA or ocular coherence tomog • Retinal detach (traction, rhegma, exud) – post, interm, or diffuse uveitis infrequent o Exud – choroidal inflame – VogtKoyanagi-Harada Syndrome, Sympathetic ophthalmia, post scleritis and orbital pseudotumor, severe retinitis or retinal vas Treatment • CS and cycloplegics – mainstay • Topical 1% prednisolone acetate – good control of ant inflame • Homatropine – prevent syn formation and reduces discomfort from ciliary spasm • Posterior sub-Tenon injection of triamcinilone acetate – superotemporal  interm, post, and diffuse uv • Oral prednisone • CS-sparing agents: methotrexate, AZT, myco mofetil, cyclosporine, tacro, cyclophos, chloramb – severe/chronic • COMPLICATIONS: o Cataract and glaucoma – most common in CS tx o Weakening of accommodation esp in <45 y/o – cycloplegics • Toxoplasmosis

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Pyrimethamine + trisulfapyrimidines (sulfadiazine, sulfamerazine, sulfamethazine) o Folinic acid (leucovorin) in dec white or platelet count o Alt: clinda + sulfonamides or minocycline • Tuberculosis o INH, ethamb, pyridoxine • Sarcoidosis o Local CS and mydriatics – active stages + sys CS like prednisone • Sympathetic ophthalmia o Local CS and cycloplegics + highdose sys CS o AZT, chloramb, cyclophos, cyclosporine ANTERIOR UVEITIS 1. Uveitis associated with joint disease • JIA Chronic Bilateral Nongranulo Iridocyclitis Girls 4-5x Detected at 5-6 y/o Insidious onset of heterchromia, anisocoria, strabismus Knee – most commonly inv joint Cardinal signs: cells and flare in the ant chamber, small to medium white keratic precip with or without fibrin flecks Band Keratopathy, secondary GL, CME Treatment:  topical CS, NSAIDs, cycloplegics  IOL implantation contraindicated • 50% ankylosing spondylitis ant uv men pain, photophobia, blurred vision limbal injection nongranulo no iris nodules Common complications: post syn, periph ant syn, cataract, GL CME is uncommon! RECURRENCE IS THE RULE! Bilateral is atypical 50% HLA-B27 (+) o

Jaybz D6 Med Class 2009

Sacroiliac radio and colonoscopy 2. Fuch’s Heterochromic Iridocyclitis (Fuch’s Uveitis Syndrome) • Uncommon • <5% • Insidious • 3rd or 4th decade • Redness, pain, photo minimal • BLURRED VISION • Small, stellate, scattered keratic precip • Gonioscopy: telangiectatic BVs • Post syn uncommon • Hypo/hyperchromia(paradoxical) • Cataract>GL • Good prognosis 3. Lens-induced Uveitis • Autoimmune • Avoid using phacotoxic uv • Hypermature cataract • Pain, photo, blurred visions • Phacolytic GL • Removal of lens material – definite tx INTERMEDIATE UVEITIS • Cyclitis, periph uv, pars planitis • CB, PP, PR, vit • Unknown cause in majority • Young adults • M=F • 80% bilateral • Floaters and blurred vision • INDIRECT ophthalmoscope and sclera depression • Snowballs/snowbanking • Post subcap cataract and CME – dec vision most common cause • CS for CME and retinal neovas POSTERIOR UVEITIS • Retina, choroid, optic nerve • Immunocompetent retinitis – toxo, syph, Behcet’s • Choroiditis – sarcoidosis, TB, V-K-H syndrome • MS – with eye pain worsened by movement • Less common: IO lymphoma, acute retin necro synd, sympa ophthal, white dot synd like multi evan white dot synd (MEWDS) or acute multifocal post placoid epitheliopathy (AMPPE) • Dx and ClinFeat o Age  < 3 y/o – masquerade syndrome – retinoblas or leukemia

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Congenital toxo, toxocariasis, syph, CMV, HSV, HZ, rubella  4-15 y/o • Toxo and toxo • Uncommon: syph, TB, sarc, Behcet’s, V-K-H  16-50 y/o • Syph, TB, sarc, toxo, Behcet’s, V-K-H, ac ret nec syndrome  > 50 y/o • Syph, TB, sarc, IO lymphoma, birdshot retinochoroiditis, ac ret nec synd, toxo, endog endoph Laterality  Unilateral • Toxo, toxoc, ac ret nec synd, endog bac or fungal Symptoms  Reduced vision  Ocular injection – eye redness is uncommon  Atypical pain Signs  Hypopyon – syph, TB, sarc, endo endoph, Behcet’s, leptosp  Ant granulo – syph, TB, sarc, V-K-H, toxo, sympa oph  Ant nongranulo – Behcet’s, ac ret nec synd, IO lymphoma, white dot GL – toxo, ac ret nec synd, sarc Vitritis – toxo, bact endoph Morph and location  Retina – toxo is most common (juxtapapillary  retinal vas) • Infectious • Rubeola or rubella – diffuse pigmentary changes of outer retina – “salt and pepper” retinopathy  Choroid • Granulomatous • TB and sarc – focal, multifocal, geog choroiditis • V-K-H synd and sympa oph – multifocal and diffuse • Birdshot and histoplasmosis – multifocal always

Jaybz D6 Med Class 2009

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o o 1. Ocular • • • • •

Optic nerve – syph, TB, sarc, toxo, MS, Lyme, IO lymphoma, sys Bartonella henselae

Trauma Sudden loss of vision – toxo, ac ret nec synd, bact endoph

toxoplasmosis Oocyst in soil or airborne in dust Undercooked meat with bradyzoites Tachyzoites – placenta Floaters and blurred vision Fluffy-white areas of focal necrotic retinochoroiditis • Elevated IgM – recently acquired • Treatment:  Small – no tx  Severe/posterior infxn – pyrimethamine and trisulfpyrimidine  Leucovorin calcium  Alt: clinda with trisulfa  Clinda causes pseudomembranous colitis  Spiramycin and minocyline also  Argon laser for subret neovas  Ant uv – topical CS and cycloplegic 2. Histoplasmosis • (+) histoplasmin • “punched-out” spots in post or periph fundus • Peripapillary atrophy and hyperpigmentation frequent • Argon laser for neovas 3. Ocular Toxocariasis • Toxocara cati or canis • Visceral larva migrans – 2y/o ave age of onset • Ocular larva migrans – 7y/o ave age of onset • Acquired thru contact with pets or pica • ClinFind  Unilateral  Red eye, blurred vision or leukocoria  Localized posterior granuloma  Peripheral granuloma – mimics snowbank  Chronic endoph  Labs: ELISA • Treatment  Systemic or periocular CS  Vitrectomy

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4. AIDS • • •

 Anthelmintics CI if only intraocular disease <100 cells/uL CD4 T lymphocyte count CMV retinitis – geog retinitis, hemorrhage in 30-40% <1% IO lymphoma

DIFFUSE UVEITIS 1. TB • Granulomatous • Giant and epitheloid cells • Caseating necrosis • Uncommon in active PTB • Tx: 3 antikochs 2. Sarcoidosis • Chronic • Granulomatous • Unknown cause • 90% pulmo inv • Uveoparotid fever/Heerfordt’s dse • Mikulicz’s syndrome – lacrimal glands inv • Retinal phlebitis • Hilar adenopathy • Elevated serum ACE, lysozyme, calcium • Tx: CS, CS-sparing 3. Syphilis • Uncommon • Widespread atrophy and hyperplasia of the RPE • VDRL or RPR • FTA-ABS or MHA-TP • Tx: aq crystalline pen G 4. Sympathetic Ophthalmia • Rare • Bilateral • Granulomatous • 10 days or years after penet eye injury; 90% within a year • Unknown cause, probably due to HPS to pigment cells of uvea • Photo, redness and blurred vision, floaters also • DALEN-FUCHS NODULES – soft yellow-white exudates in the deep layer of the retina • Serous retinal detachment • Tx: enucleation within 10 days of injury; immunosuppressives UVEITIS IN DEVELOPING COUNTRIES 1. Leptospirosis • Contact or ingestion • Fever, malaise, headache

Jaybz D6 Med Class 2009

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Renal failure and death 30% untreated • Diffuse • Hypopyon • Retinal vas • Tx: penicillin + topical CS and cycloplegics 2. Onchocerciasis • Simulium dammosum transmission • River blindness • Africa and Central America • Itching, loss of skin elasticity, depigmentation • Skin nodules • Nummular keratitis and sclerosing keratitis • Focal retinochoroiditis • Optic atrophy secondary to GL • SKIN BIOPSY • Tx: nodulectomy and ivermectin; diethylcarbamazine and suramin – toxic and used only if iver is unavail 3. Cysticercosis • Endemic in Mexico • Cysticercus cellulosae is the most common tapeworm that invades the human eye • Seizures if brain is involved • Focal calcify • Inflame reaction and fibrosis • Tx: surgical removal by pars plana vitrectomy TUMORS 1. NEVUS - freckles 2. OCULAR & OCULODERMAL MELANOCYTOSIS • Nevus of Ota 3. CHOROIDAL HEMANGIOMA • Sturge-Weber • Tx: photocoag 4. MEDULLOEPITHELIOMAS OF THE CILIARY BODY 5. MALIGNANT MELANOMA • Unilateral • Tx: enucleation • <10mm excellent prognosis 6. CHOROIDAL METASTASES • Uveal mets – most common IO tumor • Choroidal melanomas – most common primary IO tumors; women – breast most common primary site • Decreased vision, photopsia

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Jaybz D6 Med Class 2009