Signs and Symptoms and Clinical Presentations of Uveitis

ACCOMMODATIVE DEFICIENCY Sympathetic ophthalmia

ACUTE UVEITIS Behc¸et’s syndrome, VKH, toxoplasmosis, and acute multifocal placoid pigment epitheliopathy (AMPPE)

AFRICAN-AMERICAN PATIENTS Sarcoid and SLE have higher prevalence in African-American patients

AGE <20 YEARS Juvenile rheumatoid arthritis ( JRA), toxoplasmosis, toxocariasis, and masquerade (RB, leukemia, JXG)

AGE 20 TO 60 YEARS Most uveitis occurs in this age group, including HLA-B27, sarcoid, Fuchs’ heterochromic iridocyclitis (HIC), VKH, and SLE

AGE >60 YEARS Serpiginous choroidopathy, birdshot retinochoroidopathy, ARN, and masquerade (large cell lymphoma, uveal melanoma, metastatic CA, paraneoplastic)

AMBLYOPIA Toxoplasmosis or toxocariasis, causing a macular lesion

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Note: HLA are gene products derived from the major histocompatability complex (located on chromosome 6q). HLA Class I antigens (A, B, and C subtypes) are present on almost every cell and mediate antigen presentation to cytotoxic T cells (CD8+). HLA Class II antigens (DR, DP, and DQ subtypes) are present on B cells and macrophages and mediate antigen presentation to helper T cells (CD4+). The HLA-DR region appears to exert its effect on autoantibodies.

APHTHOUS ORAL ULCERS Behc¸et’s syndrome, mucocutaneous lymph node syndrome (MCLNS), OCP, SJS, SLE, HSV, Reiter’s syndrome, ulcerative colitis, and Crohn’s disease

ARTERIOLAR VASCULITIS PAN, SLE, ARN, Behc¸et’s syndrome, and idiopathic. Less common than venulitis.

ARTHRALGIAS/ARTHRITIS Ankylosing spondylitis, JRA, Behc¸et’s syndrome, sarcoidosis, Reiter’s syndrome, SLE, relapsing polychondritis, Wegener’s granulomatosis, inflammatory bowel disease (ulcerative colitis, Crohn’s disease, Whipple’s disease), RA, syphilis, brucellosis, psoriatic, Lyme disease, sporotrichosis, and gonococcal

ASIAN/MIDDLE EASTERN ANCESTRY Behc¸et’s syndrome and VKH are more prevalent in these patients

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178 UVEAL TRACT AND OCULAR INFLAMMATION

ASTHMA/WHEEZE Systemic toxocara, tuberculosis, ascariasis, aspergillosis, Churg-Strauss syndrome, and sarcoidosis

BAND KERATOPATHY JRA, sarcoidosis, multiple myeloma, and chronic uveitis in children

BILATERAL CN VII PALSY Lyme disease and sarcoidosis

CASE HISTORIES AND WORK-UP

Child with red, inflamed eye: many possible etiologies of inflammation, but always rule out RB.

Child with recurrent or chronic iridocyclitis may have hypopyon, often white-appearing eye: get ANA (on two substrates; usually positive), RF (usually negative), and HLA-B8 to rule out JRA.

Choroiditis with exudative retinal detachment and a history of episodic tinnitus: get fluorescein angiogram and audiometry, spinal tap, and MRI scanning of the brain to rule out VKH.

Elderly female with new onset ‘‘vitritis,’’ partially steroid sensitive: get

vitreal biopsy for culture, cytology, and cytokines to rule out intraocular lymphoma or infection.

Female with pars planitis and a history of episodic paresthesias: get MRI scanning of brain and spinal tap to rule out multiple sclerosis.

Granulomatous uveitis: consider chest x-ray, serum ACE, serum lysozyme, serum calcium, liver function enzymes and bilirubin, intradermal skin tests, upper body gallium scan, and chest CT scan to rule out sarcoidosis or tuberculosis.

Multifocal choroiditis, primarily posterior pole in a 35-year-old male: check HLA-A29, ERG to rule out birdshot choroiditis.

Patient with erythema nodosum, alopecia, vitiligo, poliosis: likely VKH.

Recurrent uveitis with a history of episodic diarrhea, possibly with mucus or blood in the stool: get gastroenterologist consultation with endoscopy and biopsy to rule out inflammatory bowel disease.

Recurrent uveitis with a history of low back stiffness upon awakening: get HLA-B27 and lumbosacral spine films to rule out ankylosing spondylitis.

Retinal vasculitis and a history of recurrent aphthous ulcers and pretibial skin lesions: consider biopsy of the skin lesions, HLA-B51, and HLA-B27 to rule out Behc¸et’s syndrome.

Retinal vasculitis with a history of subacute sinusitis: consider chest x-ray, sinus films, urine analysis, and serum ANCA to rule out Wegener’s granulomatosis.

Retinochoroiditis adjacent to a pigmented chorioretinal scar: get antitoxoplasma IgG and IgM antibodies to rule out toxoplasmosis.

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Unilateral iridocyclitis with fine white KP and a lighter iris in the affected eye: evaluate the lens, IOP, and gonioscopy to rule out Fuchs’ HIC.

CHILDHOOD ANTERIOR UVEITIS JRA, ankylosing spondylitis, Behc¸et’s syndrome, sarcoid, trauma, Fuchs’ HIC, HSV, and tuberculosis

CHILDHOOD INTERMEDIATE UVEITIS Pars planitis

CHILDHOOD PANUVEITIS Pars planitis, sarcoidosis, VKH, and masquerade (leukemia, retinoblastoma)

CHILDHOOD POSTERIOR UVEITIS Toxoplasmosis and toxocariasis

CHOROIDITIS, CHORIORETINITIS, RETINAL PIGMENT EPITHELIITIS VKH, serpiginous choroidopathy, sympathetic ophthalmia, multifocal chorioretinitis and panuveitis (MCP), septic multifocal choroiditis, punctate inner choroidopathy, uveal effusion, sarcoidosis, syphilis, tuberculosis, candidiasis, ocular histoplasmosis syndrome (OHS), birdshot retinochoroidopathy, acute retinal pigment epitheliitis (ARPE), AMPPE, unilateral ‘‘wipe-out’’ syndrome of Gass (diffuse unilateral subacute neuroretinitis [DUSN], toxocariasis), and CMV inclusion

CHRONIC UVEITIS JRA, birdshot retinochoroidopathy, serpiginous choroidopathy, tuberculosis, Propionibacterium acnes infection, lymphoma, sympathetic ophthalmia, sarcoidosis, and MCP

COUGH OR PULMONARY INVOLVEMENT OHS, sarcoidosis, tuberculosis, coccidiomycosis, Wegener’s granulomatosis, Pneumocystis carinii, malignancy, Churg-Strauss syndrome, and aspergillosis

DIARRHEA Inflammatory bowel disease (Crohn’s disease, ulcerative colitis, Whipple’s disease), Cogan’s syndrome, AIDS or AIDS-related complex, amoeba, mucormycosis, giardiasis, ascariasis, and schistosomiasis

EAR, AUDITORY, OR VESTIBULAR SYMPTOMS Relapsing polychondritis, Wegener’s granulomatosis, Cogan’s syndrome, VKH, sarcoidosis, syphilis, GCA, and Eales’ disease

EPIDIDYMITIS PAN and Behc¸et’s syndrome

FEMALE PREPONDERANCE JRA, AMPPE, acute zonal occult outer retinopathy (AZOOR, 90% are female), birdshot retinochoroidopathy (vitiliginous chorioretinitis), multiple evanescent white-dot syndrome

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180 UVEAL TRACT AND OCULAR INFLAMMATION

(MEWDS, 80%), punctate inner choroidopathy (PIC, 70%), subretinal fibrosis and uveitis syndrome (SFU, 100%), sarcoidosis, VKH (60%), acute macular neuroretinopathy (AMN, 95%), acute idiopathic blind spot enlargement syndrome (AIBSE, 85%), and MCP (75%)

FEVER Reiter’s syndrome, Behc¸et’s syndrome, PAN, GCA, colitis, AIDS-related complex/AIDS, Whipple’s disease, HZV, tuberculosis, brucellosis, leptospirosis, Lyme disease, candidiasis, coccidiomycosis, amoeba, mucormycosis, Cogan’s syndrome, serum sickness, trypanosomiasis, and VKH

FLU OR MONONUCLEOSIS-LIKE Histoplasmosis, leptospirosis, toxoplasmosis, CMV, AMPPE, ARPE, MEWDS, and ascariasis

GENITAL SORES OR ULCERS Reiter’s syndrome, Behc¸et’s syndrome, syphilis, and OCP

GEOGRAPHY Coccidiomycosis (U.S. Southwest desert), histoplasmosis (Mississippi River valley), Lyme disease (everywhere in the United States, especially the Northeast), and cysticercosis (Central and South America)

GLAUCOMA Posner-Schlossman syndrome (glaucomatocyclitic crisis), HSV, HZV, Fuchs’ HIC, JRA, sarcoidosis, intraocular lymphoma, VKH, sympathetic ophthalmia, Behc¸et’s syndrome, toxoplasmosis, rubella iridocyclitis, and any severe chronic iridocyclitis. However, uveitis usually has lower IOP.

HEADACHE OR MENINGITIS VKH, sarcoidosis, Behc¸et’s syndrome, cryptococcus, HSV, histoplasmosis, leptospirosis, sympathetic ophthalmia, toxoplasmosis, tuberculosis, Lyme disease, HZV, brucellosis, intraocular lymphoma, uveal effusion, Whipple’s disease, Churg-Strauss syndrome, PAN, and giardiasis

‘‘HEALTHY’’ PATIENTS DUSN, pars planitis, HSV, OHS, toxoplasmosis, toxocariasis, traumatic, Fuchs’ HIC, birdshot retinochoroidopathy, sympathetic ophthalmia, MEWDS, AMPPE, ARPE, MCP, serpiginous choroidopathy

HEMATURIA PAN, SLE, and Wegener’s granulomatosis

HEPATOSPLENOMEGALY CMV, toxocariasis, toxoplasmosis, leptospirosis, AIDS, brucellosis, and sarcoidosis

IRIS NODULES Sarcoidosis, tuberculosis, syphilis, herpetic retinitis, and leprosy

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JAUNDICE Brucellosis, schistosomiasis, leptospirosis, and CMV

MALE PREPONDERANCE HLA-B27 and Eales’ disease

MASQUERADES OF UVEITIS Congenital (PHPV, myopia), metabolic (amyloid, cholesterolosis, asteroid), vascular (Coats’ disease, CRVO, systemic hypertension), trauma (IOFB, RD, ghost cell, phacolytic, anterior segment ischemia), neoplastic (intraocular lymphoma, leukemia, retinoblastoma, MM, systemic lymphoma), and infectious disease (endophthalmitis)

MEDICATIONS Sulfa, pamidronate disodium (inhibits bone resorption in osteoporosis), rifabutin (treatment for Mycobacterium avium), and cidofovir (treatment for CMV)

NEPHRITIS Vasculitis (PAN, Wegener’s granulomatosis, etc.), Behc¸et’s syndrome, tuberculosis, sarcoidosis, toxoplasmosis, Cogan’s syndrome, cryoglobulinemia, and tubulointerstitial nephritis–uveitis (TINU) syndrome

NEURITIS, CRANIAL Syphilis, Lyme disease, sarcoidosis, subacute sclerosing panencephalitis, and Churg-Strauss syndrome

NEURITIS, PERIPHERAL Lyme disease, leprosy, HZV, sarcoidosis, multiple sclerosis, and Behc¸et’s syndrome

NIGHT SWEATS Tuberculosis, coccidiomycosis, and sarcoidosis

PERIPAPILLARY CHOROIDITIS Tuberculosis ( Jensen’s disease), toxoplasmosis, and toxocariasis

PHARYNGITIS, TONSILLITIS Sarcoidosis, viral infection, candidiasis, AIDSrelated complex/AIDS, Whipple’s disease, and gonococcal

PNEUMONIA, PNEUMONITIS CMV, AIDS-related complex/AIDS, sarcoidosis, Wegener’s granulomatosis, SLE, Whipple’s disease, coccidiomycosis, aspergillosis, and sporotrichosis

POOR HANDWASHING Toxoplasmosis toxocariasis

POSTERIOR INFLAMMATION Toxoplasmosis (18%), idiopathic vasculitis (18%), idiopathic choroiditis (18%), OHS (10%), toxocariasis (7%), CMV retinitis (6%), idiopathic retinitis (6%), serpiginous choroidopathy, AMPPE, ARN, birdshot retinochoroidopathy, leukemia, intraocular lymphoma, candidiasis, tuberculosis, and SLE

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182 UVEAL TRACT AND OCULAR INFLAMMATION

PROSTATITIS, CYSTITIS Whipple’s disease, Reiter’s syndrome, ankylosing spondylitis, and gonococcal

PSYCHOSIS VKH, sarcoidosis, Behc¸et’s syndrome, SLE, and steroids

‘‘QUIET EYE’’ JRA, pars planitis, Fuchs’ HIC, Behc¸et’s syndrome, sarcoidosis, syphilis, toxocariasis, IOFB, CMV retinitis, masquerade syndromes, and most posterior uveitic diseases

RAW MEAT Toxoplasmosis and cysticercosis

RECENT EYE SURGERY Endophthalmitis, lens-induced, sympathetic ophthalmia, and iritis from anterior segment ischemia after strabismus surgery

RETINAL ARTERIAL OBSTRUCTION FROM INFLAMMATION Collagen vascular diseases and vasculitis (PAN, SLE, GCA, Wegener’s granulomatosis, Behc¸et’s syndrome), toxoplasmosis, and Lyme disease

RETINAL PERIPHLEBITIS Tuberculosis, Eales’ disease, syphilis, sarcoidosis, and multiple sclerosis

RETINAL VASCULITIS Infectious (DUSN, HZV, syphilis, toxoplasmosis, CMV, ARN, larval embolization), immune (idiopathic, Eales’ disease, Behc¸et’s syndrome, Kawasaki disease, PAN, GCA, Wegener’s granulomatosis, pars planitis, sarcoidosis, primary Sjo¨gren’s syndrome, multiple sclerosis, SLE, Takayasu’s disease, Berger’s disease (thromboangiitis obliterans), and vascular causes (hemoglobinopathies, severe systemic hypertension, hyperviscosity syndrome, diabetes mellitus)

RETINAL ‘‘WIPEOUT’’ Bilateral acute retinal necrosis (BARN), Behc¸et’s syndrome, PAN, and DUSN

RPE DETACHMENT VKH, sympathetic ophthalmia, intraocular lymphoma, and leukemia

RPE DROPOUT Birdshot retinochoroidopathy, SFU, MEWDS, ARN, Behc¸et’s syndrome, DUSN, and PAN

RPE HYPERPLASIA RP, syphilis, PIC, ARPE, VKH, and AMPPE

SADDLE NOSE Syphilis (congenital), Wegener’s granulomatosis, and relapsing polychondritis

SCLERITIS, DIFFUSE Vasculitis (RA, Wegener’s granulomatosis, SLE, PAN, relapsing polychondritis, Takayasu’s disease), syphilis, HSV, HZV,

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Reiter’s syndrome, VKH, toxoplasmosis, tuberculosis, inflammatory bowel disease, Behc¸et’s syndrome, sarcoidosis, leprosy, mumps, and masquerade (melanoma)

SCLERITIS, SECTORAL Tuberculosis, gout, and syphilis

SECTORAL IRIS ATROPHY HSV, HZV, previous attacks of angle closure glaucoma, and Fuchs’ HIC

‘‘SICK ANIMAL’’ EXPOSURE Brucellosis and trichinosis

SINUSITIS Wegener’s granulomatosis, sarcoidosis, Whipple’s disease, Churg-Strauss syndrome, relapsing polychondritis, and mucormycosis

SKIN NODULES Behc¸et’s syndrome, ulcerative colitis, histoplasmosis, sarcoidosis, Crohn’s disease, AMPPE, leprosy, and onchocerciasis

SKIN RASH Behc¸et’s syndrome, syphilis, Lyme disease (erythema chronicum migrans), rubella, rubeola, Reiter’s syndrome (keratoderma blennorrhagicum), leprosy, toxoplasmosis, SLE, ulcerative colitis, sarcoidosis, SJS, mucocutaneous lymph node syndrome, psoriatic, giardiasis, loiasis, schistosomiasis, and HZV

‘‘SPILLOVER’’ IRIDOCYCLITIS Pars planitis, VKH, toxoplasmosis, toxocariasis, candidiasis, and herpetic retinitis

SPLENDORE-HEPLIN PHENOMENON Histopathologic finding of a IgM, IgA, and IgG reaction with eosinophils in a ring around a parasite

SUBRETINAL FIBROSIS SFU, ICSR, age-related macular degeneration (AMD), large nevi, melanoma, serpiginous choroidopathy, sarcoidosis, and OHS

SUBRETINAL NEOVASCULARIZATION (CNVM) OHS, toxoplasmosis, Behc¸et’s syndrome, birdshot retinochoroidopathy, sarcoidosis, choroiditis, toxocariasis, VKH, rubella, serpiginous choroidopathy, and coccidiomycosis

‘‘SUNSET GLOW’’ FUNDUS VKH and syphilis

SYNECHIAE JRA, syphilis, VKH, Behc¸et’s syndrome, Reiter’s syndrome, psoriasis, sarcoidosis, pars planitis, HZV, ankylosing spondylitis, and SFU

UNPASTEURIZED MILK Tuberculosis and brucellosis

UNPROTECTED SEX HIV, HSV, CMV, and syphilis

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