188 UVEAL TRACT AND OCULAR INFLAMMATION

PANUVEITIS Behc¸et’s syndrome, sympathetic ophthalmia, sarcoidosis, VKH, syphilis, and tuberculosis

PHACOANTIGENIC UVEITIS, PHACOANYPHYLACTIC Lens proteins leak through ruptured capsule, causing zonal granulomatous inflammation, usually following trauma or surgery; 3 to 7% coincidence with sympathetic ophthalmia. Treat by cataract extraction.

POSNER-SCHLOSSMAN SYNDROME Glaucomocyclitic crisis: iritis, increased IOP, and corneal edema

POSTSURGICAL May be normal inflammation, lens-induced, or infectious

POSTERIOR UVEITIS SPILLOVER Candidiasis, toxoplasmosis, or toxocariasis

SCHWARTZ SYNDROME Acute anterior uveitis from chronic RRD

Intermediate Uveitis Diagnosis and Work-Up

General: Intermediate uveitis comprises 15% of all cases of uveitis. Patients typically present asymptomatic with a white eye or with floaters and blurred vision from vitreous cells. Patients also may have mild anterior segment inflammation, retinal vessel sheathing, pars plana snowbanking, inferior retinal ‘‘snowballs,’’ CME, cotton-wool spots, PSC cataract, vitreous hemorrhage, or RD.

INTERMEDIATE GRANULOMATOUS UVEITIS WITH LOCAL DISEASE

Infectious: toxocariasis (consider Toxocara titer)

Autoimmune: pars planitis (consider ESR)

Masquerade: IOFB (consider ultrasound)

INTERMEDIATE GRANULOMATOUS UVEITIS WITH SYSTEMIC DISEASE

Infectious: Lyme disease

Autoimmune: sarcoidosis and multiple sclerosis

INTERMEDIATE NONGRANULOMATOUS UVEITIS WITH LOCAL DISEASE

Autoimmune: Fuchs’ HIC, pars planitis, and idiopathic senile vitritis (look for heterochromia, TID, and angle vessels)

Masquerade: ophthalmia nodosa (tarantula hair) and familial exudative vitreoretinopathy

INTERMEDIATE NONGRANULOMATOUS UVEITIS WITH SYSTEMIC DISEASE

Infectious: Lyme disease (consider Lyme titers and western blot)

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Autoimmune: multiple sclerosis (consider MRI, neurologic exam)

Masquerade: amyloid

Intermediate Uveitis Diseases

PARS PLANITIS Forty-five percent of intermediate uveitis is caused by pars planitis. It is usually seen in children and young adults, with uveitis of unknown etiology that is bilateral in 75% of cases. Patients may be asymptomatic or have floaters or decreased VA, usually from CME. They may also have band keratopathy, low-grade AC cell with endothelial dusting, numerous vitreous cells, ‘‘snowbanking’’ (white aggregates composed of glial elements and fibrovascular tissue), cyclitic membrane, disk edema with diffuse leakage on FA and staining, and peripheral retinal NV (poorer prognosis). There is a smoldering course without exacerbations in 59% of cases, with exacerbations in 31%, and a benign self-limited course in 10%. Associated with cataract (42%), chronic CME (28%), and RD (5%).

Most cases are idiopathic but may be autoimmune or familial. HLADR2 in 67% of cases; also found in 50 to 70% of multiple sclerosis patients versus 20 to 25% of normal controls. There is a 20% 5-year incidence of multiple sclerosis.

No treatment (do not treat every AC cell). Treat CME with steroids or carbonic anhydrase inhibitors. May laser or cryotherapy the pars plana membrane, especially if there is NV. Rule out secondary causes.

CHRONIC CYCLITIS Same as pars planitis but without exudate; work-up with CXR, ACE, and FTA-Abs.

IDIOPATHIC AGE-RELATED VITRITIS Age of onset: 60 to 70 years. Consider large cell lymphoma (needs diagnostic vitrectomy and DNA PCR), Whipple’s disease, syphilis, pars planitis, tuberculosis, and sarcoidosis. Steroids are ineffective.

LYME DISEASE Spirochete Borrelia burgdorferi carried by Ixodes dammini tick found on rodents, deer, birds, cats, and dogs, usually in early summer to midautumn. Causes up to 3% of cases of intermediate uveitis.

Three stages:

Stage 1: erythema chronicum migrans, follicular conjunctivitis, headache, malaise, myalgia, arthralgias, and fever

Stage 2: follows in 1 to 4 months with neurologic (in 30 to 40% of cases; usually CN VII palsy, encephalitis, or meningitis), musculoskeletal (arthritis, tendonitis), cardiac (myocarditis, heart block), and ophthalmic symptoms (keratitis, iritis, intermediate uveitis, vitritis, panophthalmitis, or optic neuritis)

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190 UVEAL TRACT AND OCULAR INFLAMMATION

Stage 3: >5 months; shows chronic atrophic skin changes, keratitis, chronic meningitis, chronic arthritis, and respiratory distress.

Diagnose with enzyme-linked immunosorbent assay (ELISA) IgM and IgG (negative in early stage 1) and immunofluorescent antibody titers; beware of false (þ) FTA-Abs.

Treat with tetracycline, erythromycin, penicillin, or intravenous ceftriaxone or penicillin if there is CNS involvement.

OTHERS Fuchs’ HIC (27%), sarcoidosis (14%), multiple sclerosis (5%), toxoplasmosis, toxocariasis, and tuberculosis

Posterior Uveitis Diagnosis and Work-Up

General: Posterior uveitis represents 38% of all cases of uveitis. Patients usually present with decreased VA and may have pain, floaters, metamorphopsia, and systemic symptoms. There are many etiologies, but the most common are toxoplasmosis (18%), idiopathic vasculitis (18%), idiopathic choroiditis (10%), multifocal choroiditis from tuberculosis or syphilis, histoplasmosis (10%), and toxocariasis (7%).

POSTERIOR GRANULOMATOUS UVEITIS WITH LOCAL DISEASE

Infectious: toxoplasmosis, OHS, BARN (HZV, HSV-2), and toxocariasis

Autoimmune: sympathetic ophthalmia and pars planitis (consider ultrasound, FA)

Masquerade: intraocular lymphoma

POSTERIOR GRANULOMATOUS UVEITIS WITH SYSTEMIC DISEASE

Infectious: CMV, tuberculosis, syphilis, toxoplasmosis, brucellosis, coccidiomycosis, helminthic, ascariasis, onchocerciasis, microfilaria, cysticercosis, and schistosomiasis (consider tuberculosis, VDRL, FTA-Abs, chest x-ray, Toxocara titer, histoplasmosis or coccidio skin test, and eosinophils)

Autoimmune: sarcoidosis and VKH (consider ACE, calcium, chest x-ray, gallium scan, anergy, and conjunctival biopsy; look for poliosis, vitiligo, and hearing problems)

Masquerade: fungal endophthalmitis (candidiasis, sporotrichosis, aspergillus) and amyloid

POSTERIOR NONGRANULOMATOUS UVEITIS LOCAL OCULAR DISEASE

Trauma: radiation vasculitis

Infectious: CMV, HZV (BARN), DUSN, ophthalmomyiasis, and rubella (consider eosinophils, serum glutamic-oxaloacetic transaminase [SGOT], lactate dehydrogenase [LDH], FA, and HIV)

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Autoimmune: MEWDS, AMPPE, punctate inner choroidopathy, subretinal fibrosis and uveitis syndrome, birdshot retinochoroidopathy, acute retinal pigment epitheliitis, MCP, serpiginous choroidopathy, and posterior scleritis (consider FA, ERG, electro-olfactogram [EOG], hepatitis B virus surface antigen, HLA-A29, vitiligo, Epstein-Barr virus [EBV] titers with ELISA assay and IgM)

Masquerade: retinitis pigmentosa, amyloid, and tumors

POSTERIOR NONGRANULOMATOUS UVEITIS WITH

SYSTEMIC DISEASE

Infectious: syphilis, HSV, HZV, Lyme disease, CMV, trypanosomiasis, rubella, measles, Whipple’s disease, Acanthamoeba, and giardiasis

Autoimmune vasculitis: Behc¸et’s syndrome, SLE, relapsing polychondritis, Crohn’s disease, Wegener’s granulomatosis, PAN, scleroderma, dermatomyositis, cryoglobulinemia, Sjo¨gren’s syndrome, Eales’ disease, and multiple sclerosis (consider ANA, RF, cryoglobulins, ESR, ANCA, CIC, tuberculosis, spinal tap, SPEP, hepatitis B surface antigen, chest x-ray, sinus, l-acid-glycoprotein, and properdin factor B)

Masquerade: intraocular lymphoma, endogenous endophthalmitis, familial exudative vitreoretinopathy, and leukemia (consider CBC)

Posterior Uveitis Diseases

ACUTE MACULAR NEURORETINOPATHY (AMN) Rare bilateral white-dot syndrome that affects young healthy adults with posterior pole middle and outer retinal lesions.

ACUTE MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY (AMPPE) Acute illness that is postviral in one third of patients, usually seen in young females (average age: 25) who present with a headache and variably decreased VA; bilateral (maybe weeks later). Exam shows multiple deep, large (1 DD) white placoid RPE lesions in the posterior pole and vitreous cells in 50%. On FA, blocks early from obliterative choroidal vasculitis and stains late (hyperfluorescent). The early multifocal hypofluorescence looks like hypertensive Elschnig’s choroidal infarctions. Has full recovery; 80% are 20/40 or better, but may leave permanent RPE disturbance. No treatment necessary (rarely has CNVM, CME, cerebral vasculitis, or pleocytosis).

ACUTE RETINAL PIGMENT EPITHELIITIS (ARPE), KRILL’S DISEASE Usually seen in young adults with decreased VA and gray macular spots with yellow-white halos, which resolve in 6 to 12 weeks.

ACUTE ZONAL OCCULT OUTER RETINOPATHY (AZOOR) Very rare whitedot syndrome of young women with damage of outer retinal layers without

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192 UVEAL TRACT AND OCULAR INFLAMMATION

noticeable fundus changes but with progressive VF loss, photopsias, and central distortion. FA shows confluent hyperfluorescence with granularity. Has ERG changes.

BIRDSHOT RETINOCHOROIDOPATHY, VITILIGINOUS CHORIORETINITIS

Seen in older (average age: 51) women with bilateral decreased VA, floaters, photopsias, and nyctalopia. Exam shows numerous vitreous cells and cream-colored depigmented large (1000 mm) lesions throughout the fundus. CME present in 50% with disk staining. Typically a chronic course, with late disk pallor and vessel attenuation. HLA-A29.2 is positive in 96% of cases (highest known HLA-associated disease; normal population is 7% positive). Thought to be a response to retinal S antigen (arrestin). May treat CME with steroids.

DIFFUSE UNILATERAL SUBACUTE NEURORETINITIS (DUSN) Nematode migration under retina often from parasite carried by raccoons.

EALES’ DISEASE Rare, idiopathic, bilateral occlusive peripheral retinal vasculitis (possible allergy to tuberculoprotein) affecting 20to 40-year-old males in India and the Middle East. Presents with floaters or blurring, retinal nonperfusion, NV, vitreous hemorrhage (VH), vessel sheathing, and tortuosity; often have branch retinal vein occlusion (BRVO), and occasionally have vestibulo-auditory symptoms. No specific therapy; 54% end with VA 20/20–20/50.

MULTIFOCAL CHORIORETINITIS AND PANUVEITIS (MCP) Older age, bilateral white-dot syndrome with marked AC and vitreous cell, multiple 50–200 mm lesions (smaller than AMPPE) in the posterior pole (more punched out and discrete than birdshot retinochoroidopathy) that block early and stain late. Chronic course with CME, CNVM (present in 45% of cases, the major cause of vision loss). The second eye may not be involved until years later. Treat with steroids or cyclosporine, but expect poor visual prognosis.

MULTIPLE EVANESCENT WHITE-DOT SYNDROME (MEWDS) Young (average age: 28) myopic women (75%) without viral prodrome but with good VA, unilateral shimmering photopsias, and paracentral scotomas or enlarged blind spot. Exam shows multiple 100–200 mm punctate perifoveal lesions with macular granularity and possibly a few vitreous cells. ‘‘Wreath’’ sign seen on FA with lesions that hyperfluoresce early and stain late (also late disk staining). Good prognosis; self-limited course lasts 7 weeks; no therapy.

PUNCTATE INNER CHOROIDOPATHY (PIC) May be a subgroup of MCP (similar HLA association) in young myopic females without vitreous cells

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but with small dots in the posterior pole. Benign course, less recurrence than MCP.

SERPIGINOUS CHOROIDOPATHY Seen in middle-aged (40 to 50 years old) Caucasian patients with bilateral chronic chorioretinitis starting at the ON and extending through the posterior pole (predilection for the macula) with vitreous cells. Lesions wax and wane, lasting months, then regress, leaving overlying RPE loss, and new lesions form at border of old lesions. FA blocks early and stains late with pathognomonic hyperfluorescent ‘‘brushfire border.’’ Indolent course; steroids 1 mg/kg have not proven beneficial, and immunosuppressives do not work well. Twenty-five percent of cases have CNVM.

SUBRETINAL FIBROSIS AND UVEITIS SYNDROME (SFU) Very rare, chronic, bilateral disease of young healthy, female, usually AfricanAmerican patients with multifocal choroiditis that progresses to subretinal plaques, with profound vision loss and poor visual prognosis.

TOXOPLASMOSIS Protozoan Toxoplasma gondii infection with moderate to severe vitritis and focal white necrotizing retinitis. Typically retinitis reactivates next to an old pigmented scar. Fifty percent of cases have ‘‘mutton-fat’’ granulomatous KP and AC cell. Subnormal ERG; may develop retinal NV or CNVM. Usually due to reactivation of congenitally acquired toxoplasmic cysts latent in retina that were acquired early in pregnancy, often with bilateral retinochoroiditis with macular predilection. May cause 30 to 50% of posterior uveitis. Very common in developing countries, and decreases cataract surgical success rate there by 20%.

Organism is highly prevalent in North America. Obligate intracellular parasite in cats (the definitive host) that shed oocysts in their feces. Cysts may remain viable for up to 1 year. Humans are intermediate host, usually from ingestion of oocysts in contaminated food (especially raw meat) or soil (such as cat litter), resulting in human infection with predilection for the retina and other CNS structures.

Congenital: transplacental, excavated macular scar, hydrocephaly, seizures, cerebral calcifications, jaundice, and hepatosplenomegaly

Acquired: only 10 to 20% are symptomatic with febrile illness. In AIDS patients, is usually newly acquired, and 30 to 50% have toxoplasmic encephalitis; thus, order CT.

Diagnose clinically, plus order serum antibodies (any titer is signficiant in the presence of a fundus lesion). Also may use Sabin-Feldman dye test. Associated with Fuchs’ HIC.

Many cases resolve and do not need treatment. However, in general, treat the active infection if it is within the arcades or peripapillary, large (>1 DD) lesion, or has severe vitritis. Do not treat during pregnancy. Mepron (Atovarone) may be available for monotherapy

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