Uveitis and common variable immunodeficiency: data from the DEF-I study and literature review.

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.