Purpose: To examine the relation between uveitis and common variable immunodeficiency (CVID).

Methods: Retrospective analysis of patients included in the French DEFI cohort of adults with CVID and of patients identified by reviewing the literature.

Results: Four patients were identified in the DEFI study (frequency of uveitis: 1.6%). The course of uveitis was not changed in the patients who started intravenous immunoglobulins replacement therapy after CVID diagnosis. Ten cases of CVID-associated uveitis were listed in the literature. Overall, among the 14 patients, uveitis was always chronic, usually bilateral (n = 11) and granulomatous (n = 9). Seven patients presented with a “sarcoid-likeâ syndrome. Nine patients had granulomatous uveitis suggestive of ocular sarcoidosis. Five patients were treated with local corticosteroids, and 9 required systemic treatment (corticosteroids alone n = 5 and/or immunosuppressive agents n = 4).

Conclusions: CVID can be associated with uveitis. Since uveitis can reveal the immunodeficiency, CVID diagnosis should be considered at the time of uveitis diagnosis.