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

Ocul Immunol Inflamm. 2012 Jun;20(3):163-70. doi: 10.3109/09273948.2012.674612. Epub 2012 Apr 17.

Abstract

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.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Clinical Trials as Topic
  • Common Variable Immunodeficiency / complications
  • Common Variable Immunodeficiency / diagnosis*
  • Common Variable Immunodeficiency / drug therapy
  • Female
  • France
  • Granulomatous Disease, Chronic / diagnosis
  • Granulomatous Disease, Chronic / drug therapy
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Multicenter Studies as Topic
  • Retrospective Studies
  • Sarcoidosis / diagnosis
  • Sarcoidosis / drug therapy
  • Uveitis / diagnosis*
  • Uveitis / drug therapy
  • Uveitis / etiology
  • Young Adult

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents