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Erica Z. Oltra, Cody Morris, Andrea D. Birnbaum, Howard H. Tessler, Debra A. Goldstein; Chronic Granulomatous Uveitis in Common Variable Immunodeficiency. Invest. Ophthalmol. Vis. Sci. 2011;52(14):4281.
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To describe three cases of chronic granulomatous anterior uveitis in patients with Common Variable Immunodeficiency (CVID) and to present a literature review of the incidence and pathogenesis of autoimmune and sarcoid-like diseases in patients with CVID.
A retrospective review was performed of patients with uveitis and CVID seen at the uveitis service of the University of Illinois Chicago and the private office of one of the authors. Data collected included medical and ocular histories, ophthalmic findings, management and visual outcomes. An English language Medline search was also performed for all cases in the literature of ophthalmologic diseases in patients with CVID.
We identified three patients with uveitis and CVID. The first patient, a 53-year-old Caucasian Female, complained of floaters, and was found to have anterior chamber cells, Koeppe nodules, granulomatous keratic precipitates, vitreous cells and cystoid macular edema. She carried a diagnosis of CVID, and was also found to have elevated ACE and lysozyme. She responded well to topical corticosteroids, but was lost to follow up after 4 months. The second patient is a 53-year-old Caucasian Female with CVID and blurred vision. She was found to have granulomatous keratic precipitates and anterior chamber cells and flare. She was diagnosed with granulomatous anterior uveitis and initially responded to topical steroids, but her course was complicated by recurrent uveitis despite therapy with systemic immunomodulators. The third patient is an 11-year-old Caucasian Female with Juvenile Idiopathic Arthritis (JIA) diagnosed at 17 months of age, and on treatment for asymptomatic chronic anterior uveitis since 18 months of age. She was diagnosed with CVID at 5 years of age. The disease has remained quiescent on topical corticosteroids and systemic immunomodulators for persistent uveitis and joint disease.
There are few cases of ophthalmologic diseases associated with CVID reported in the literature, and even fewer cases of patients with uveitis. All three patients in this series had chronic granulomatous anterior uveitis with no posterior involvement. The two adult patients were symptomatic, while the child's uveitis was picked up on screening because of the diagnosis of JIA. Physicians caring for patients with CVID should be aware of the association with systemic inflammatory disease and uveitis.
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