Intermediate Uveitis
Andrea D. Birnbaum
Anjum F. Koreishi
Debra A. Goldstein
Intermediate uveitis (IU) refers to ocular inflammation that localizes primarily to the vitreous and peripheral retina.
Epidemiology and Etiology
• IU most often occurs in children and young adults.
• IU accounts for up to 15% of uveitis cases in adults, with higher percentages reported at tertiary referral centers, and it accounts for up to 28% of uveitis cases in children.
• No clear gender predilection, but a recent report suggests that IU is more common in females.
• Human leukocyte antigen (HLA) associations reported, specifically HLA-DR15, HLA-DR17, and HLA-A28.
HLA-DR15 is also associated with multiple sclerosis.
Symptoms
• Blurred vision, floaters
• Pain and photophobia only if concomitant anterior segment inflammation, which is more common in young children.
Signs
The American Uveitis Society describes IU as ocular inflammation localized primarily to the vitreous. Associated findings may include the following:
• Bilateral involvement in 75% of patients.
• Cystoid macular edema (CME) (Fig. 6-3A-C)
• Retinal vasculitis may involve the posterior pole or may be limited to periphery (Figs. 6-3D, 6-4, 6-5 and 6-6).
Patients may develop peripheral retinal and/or anterior segment neovascularization and vitreous hemorrhage as a result of peripheral ischemia.
• There is no clear data suggesting that limited peripheral vasculitis requires treatment. Anterior uveitis may also be present, with cells, keratic precipitates, and band keratopathy.
Differential Diagnosis
• Pars planitis accounts for 50% of IU, is idiopathic, and usually presents between 5 and
35 years of age. It has no extraocular manifestations and is a diagnosis of exclusion.
35 years of age. It has no extraocular manifestations and is a diagnosis of exclusion.
• Sarcoidosis is the most common identifiable etiology.
• Multiple sclerosis typically presents in females aged 20 to 50 years. Multiple sclerosis-associated IU is more frequently associated with retinal periphlebitis. Tumor necrosis factor (TNF) inhibitors are contraindicated.
• Inflammatory bowel disease can cause IU and may be associated with vasculitis and choroiditis.
• Intraocular lymphoma (Fig. 6-7) displays various ocular manifestations. This masquerade is typically seen in older patients, but they may be middle aged.
• Syphilis should be in the differential for all forms of uveitis.
• Tuberculosis typically causes a granulomatous uveitis and choroiditis.
• Toxocariasis is typically unilateral and can cause dense vitritis in ˜25% of cases.
• Bartonellosis is associated with cat licks/scratches, and can cause IU. Common manifestations include multifocal retinitis with or without optic nerve edema and macular star.
• Other etiologies include Lyme disease, Whipple disease, and T-cell leukemia-lymphoma virus type 1.
Diagnostic Evaluation
• Etiology assessment:
Sarcoidosis: angiotensin-converting enzyme (ACE) (unless patient on ACE inhibitor), lysozyme, and chest X-ray, or computed tomography.
Syphilis: specific treponemal test (fluorescent treponemal antibody absorption, Treponema pallidum immunoglobulin G, microhemagglutination assay for Treponema pallidum antibodies) or non-treponemal tests such as RPR or VDRL. Typically, T. pallidum IgG with reflex RPR is performed.
Tuberculosis: Interferon gamma release assay (such as QuantiFERON Gold), or tuberculin skin test (such as the purified protein derivative).
Multiple sclerosis: magnetic resonance imaging (MRI) brain and orbit, if indicated by history or review of systems or if considering TNF-inhibitor therapy.
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