184 UVEAL TRACT AND OCULAR INFLAMMATION

URETHRAL DISCHARGE Reiter’s syndrome, chlamydia, syphilis, HSV, and gonococcal

VASCULITIS, CEREBRAL AMPPE, Behc¸et’s syndrome, HSV, syphilis, and Lyme disease

VIRAL ETIOLOGY OF RETINAL DISEASE AMPPE, MEWDS, and Leber’s stellate neuroretinitis

WHITE-DOT SYNDROMES THAT ARE UNILATERAL MEWDS and DUSN

WHITE-DOT SYNDROMES WITH GOOD PROGNOSIS AMPPE, ARPE,

PIC, and MEWDS

WHITE-DOT SYNDROMES WITH GUARDED PROGNOSIS (may have CNVM or chronic course) Birdshot retinochoroidopathy, DUSN, MCP, and serpiginous choroidopathy

WHITE-DOT SYNDROME MASQUERADES Sarcoid, B-cell lymphoma, sympathetic ophthalmia, and VKH

CAUCASIAN PATIENTS HLA-B27 and multiple sclerosis

Anterior Uveitis Diagnosis and Work-Up

General: Anterior uveitis accounts for 28% of all cases of uveitis.

Signs and symptoms: acute pain, blurred vision, photophobia, red eye with ciliary flush (except JRA), hypotony (if increased IOP, consider HSV), AC cell, KP (granulomatous type has mutton-fat KP, epithelioid or giant cells, and Arlt’s triangle on inferior cornea secondary to convection currents), which may spill into anterior vitreous, hypopyon (if in a quiet eye, consider masquerade), iris atrophy, and Koeppe or Busacca iris nodules. Chronic iridocyclitis: lymphocytes and plasma cells (Russell’s bodies), isolated or part of ankylosing spondylitis, psoriatic arthritis, inflammatory bowel disease, Fuchs’ HIC, JRA, phacoanaphylaxis, and sarcoidosis.

Basic work-up: order MHA-TP or FTA, CBC, electrolytes, and chest x-ray, then add other tests as suspicion warrants.

Goodman, Ophtho Notes © 2003 Thieme

All rights reserved. Usage subject to terms and conditions of license.

ANTERIOR UVEITIS, GRANULOMATOUS WITH LOCAL OCULAR DISEASE

Trauma

Infectious: chronic anaerobic endophthalmitis, HSV, HZV, onchocerciasis, and schistosomiasis

Autoimmune: sympathetic ophthalmia, lens-induced (phacoanaphylactic), and Fuchs’ HIC. Consider HLA-A11, S-Ag, vitreous culture, and look for Dalen-Fuchs nodules.

Masquerade: juvenile xanthogranuloma

ANTERIOR UVEITIS, GRANULOMATOUS WITH SYSTEMIC DISEASE

Infectious: syphilis, tuberculosis, toxoplasmosis, leprosy, brucellosis, and helminthic. Consider PPD, VDRL, FTA-Abs, eosinophil count, and Cowdry-A test.

Autoimmune: sarcoidosis and VKH. Consider ACE, calcium, anergy, chest x-ray, conjunctival biopsy, gallium scan, poliosis, vitiligo, and hearing exam.

Masquerade: intraocular lymphoma and leukemia

ANTERIOR UVEITIS, NONGRANULOMATOUS WITH LOCAL DISEASE

Trauma: surgical or nonsurgical, UGH syndrome, graft versus host disease, and polypropylene reaction

Infectious: HSV, HZV, and Staphylococcus aureus

Autoimmune: Fuchs’ HIC, lens-induced (phacogenic), glaucomatocyclitic crisis, and idiopathic

Masquerade: juvenile xanthogranuloma, tumor, and pigment dispersion syndrome

ANTERIOR UVEITIS, NONGRANULOMATOUS WITH SYSTEMIC DISEASE

Infectious: syphilis, HSV, HZV, Lyme disease, leptospirosis, amoeba, and giardiasis. Consider VDRL, FTA-Abs, anergy panel, stool for amoeba, Lyme serology, and corneal sensitivity.

Autoimmune with joint involvement: JRA, HLA-B27, ankylosing spondylitis, psoriatic, inflammatory bowel disease, Reiter’s syndrome, and Sjo¨gren’s syndrome. Consider sacroiliac x-rays, ANA for JRA, RF, ESR, uric acid, VDRL, FTA-Abs (MHA-TP), HLA-B27, ear biopsy, Schirmer’s testing, and salivary biopsy.

Autoimmune with vasculitis: Behc¸et’s syndrome, PAN, relapsing polychondritis, serum sickness, mucocutaneous lymph node syndrome, and rarely RA. Consider CRP, ESR, cryoglobulins, C3, C4, CH50, circulating immune complexes (CIC), skin biopsy, eosinophils, ANA, RF, ANCA, chest x-ray, tuberculosis skin, urinanalysis, properdin factor B, serum lysozyme, and 1-acid glycoprotein.

Goodman, Ophtho Notes © 2003 Thieme

All rights reserved. Usage subject to terms and conditions of license.

186 UVEAL TRACT AND OCULAR INFLAMMATION

Anterior Uveitis Diseases

BRUCELLOSIS Acquired from undercooked animal meat, causing an acute febrile illness with possible iridocyclitis; treat with tetracycline.

FUCHS’ HETEROCHROMIC IRIDOCYCLITIS (HIC) (9% of anterior uveitis)

Unilateral mild iritis with heterochromia from stromal thinning (darker irides become lighter and vice versa), diffuse fine stellate KP, PSC cataract, glaucoma (15%), and rubeosis or vessels crossing the TM that may lead to hyphema. Associated with chorioretinal scars (toxoplasmosis with possible reaction to previous infection).

HALLERMANN-STREIFF SYNDROME Characterized by skin disease, alopecia, short stature, congenital cataract, glaucoma (PAS and posterior synechiae), and severe granulomatous uveitis, with onset usually at about age 20.

HERPES SIMPLEX VIRUS (HSV) (6%) Keratouveitis often with increased IOP from trabeculitis.

HLA-B27 RELATED (11%) Most common identifiable cause of acute anterior uveitis.

Anklyosing spondylitis, 5% of cases of acute anterior uveitis: acute, recurrent, usually unilateral uveitis with fibrinous AC reaction, typically in males with low back pain from spondyloarthritis (x-ray the sacroiliac joints) or pauciarticular arthritis; 5% of patients have aortitis that may lead to aortic insufficiency. Average age of onset is 9 years. Uveitis may be prodrome for systemic disease. Ninety-five percent of cases are HLA-B27 positive.

Reiter’s syndrome: classic triad of bilateral papillary conjunctivitis, nongonococcal urethritis, and arthritis (mnemonic: can’t see, can’t pee, can’t climb a tree). Often have a conjunctival mucopurulent discharge, corneal punctuate epithelial erosions (PEE), and punctate SEI or infiltrate, and may have uveitis, keratoderma blennorrhagicum (red, scaly rash on palms and soles), and circinate balanitis. Age of onset: 16 to 40 years; 75 to 95% of cases are HLA-B27 positive. May result from immunologic reaction to infectious antigen (Chlaymdia, Yersinia, Campylobacter, Shigella, etc.).

Psoriasis: 10% of patients have iritis but only if joints are involved (20% of psoriatic patients).

Inflammatory bowel disease (ulcerative colitis, Crohn’s disease, Whipple’s disease): mild iritis that coincides with bowel symptoms; 75% of cases are B27 positive.

IDIOPATHIC (43%) No work-up needed if it is first episode, mild, and unilateral.

Goodman, Ophtho Notes © 2003 Thieme

All rights reserved. Usage subject to terms and conditions of license.

JUVENILE RHEUMATOID ARTHRITIS (JRA) (10%) Chronic synovitis with extraarticular manifestations such as chronic iridocyclitis. Typically involes white eye and is bilateral and nongranulomatous; associated with cataract (60%), glaucoma (22%), and band keratopathy (50%). JRA is usually seen in females (80%), 4 to 6 years old; 80% of patients who are ANA positive develop anterior uveitis. If untreated, JRA will lead to decreased VA within 2 years. Ocular symptoms may persist into adulthood even though joint symptoms resolve.

Four types:

Type 1: most common iritis; pauciarticular (four or fewer joints involved), early onset (25 to 40% of JRA), large joint disease. Ninety-five percent of patients who are ANA positive and RF negative have uveitis.

Chronic iridocyclitis of young girls: same as type 1 but no joint involvement

Type 2: boys with pauciarticular B27þ

Type 3: polyarticular (40% of JRA)

Type 4: severe systemic JRA, Still’s disease

Treat iritis initially with hourly steroids and cycloplegic if case is mild or with atropine if severe. Consider steroid ointment at bedtime (qhs), NSAID, and immunosuppressives. Must eliminate all cell (is a very ‘‘sticky’’ inflammation) and treat cell, not flare. Once quiet, usually see patients every 3 months if ANA positive or yearly if negative; follow slit lamp exam to rule out posterior synechiae.

MASQUERADES OF ANTERIOR UVEITIS Intraocular lymphoma, leukemia, postoperative endophthalmitis (consider vitreous biopsy or tap), RB, chronic RD, retained IOFB, and melanoma

MEDICATIONS Cobalt (tattoos), oral contraceptives, quinidine, sulfa, Xalatan, topical beta-blockers, diethylcarbamazine (treatment for loiasis), MMC, cidofovir (for CMV retinitis), rifabutin (prophylaxis against Mycobacterium avium complex in AIDS patients; may cause severe uveitis with hypopyon when used with clarithromycin or fluconazole).

OCULAR OR ANTERIOR SEGMENT ISCHEMIA Scleral buckle procedure, multiple muscle surgery, carotid disease (chronic inflammation with few KP, poorly reactive pupil), and GCA. Treat with steroids, cycloplegics.

ONCHOCERCIASIS Onchocerca volvulus microfiliarial death in eye incites inflammatory response.

OTHER VIRAL CAUSES OF UVEITIS HZV (40% of patients develop uveitis), CMV, HIV, and influenza

Goodman, Ophtho Notes © 2003 Thieme

All rights reserved. Usage subject to terms and conditions of license.