Resolution of Juvenile Idiopathic Arthritis-Associated Uveitis after Development of Common Variable Immunodeficiency

Abstract

Purpose

To describe the occurrence of common variable immunodeficiency (CVID) in a patient with juvenile idiopathic arthritis (JIA) and JIA-associated uveitis.

Methods/Results

Case report. A 29-year-old woman was followed-up since the age of 10 years because of right eye JIA-associated recurrent anterior uveitis. She was treated with steroids and immunosuppressants with good control of uveitis and arthritis. At the age of 17 years, she did not experience any further relapse of uveitis or arthritis and both diseases were considered to be in remission. Concomitantly, she started to have recurrent infections and later she underwent splenectomy because of autoimmune hemolytic anemia and thrombocytopenia. Liver biopsy disclosed granulomatous hepatitis. She was ultimately diagnosed with CVID at the age of 23 years when her blood tests revealed neutropenia and severe panhypogammaglobulinemia. She has been treated since then with intravenous immunoglobulins with good control of the disease. Since the development of CVID, she has had no relapses of uveitis or arthritis during a follow-up period of 12 years.

Conclusions

Common variable immunodeficiency (CVID) is the most common primary immunodeficiency where defective antibody formation is the most common feature with B-cell differentiation failure. Ocular complications have been rarely documented and included bacterial conjunctivitis, retinal vasculitis and multifocal choroiditis. We herein report on the occurrence of JIA-associated uveitis as a comorbid manifestation of CVID.

We speculate a role for B cells in the pathogenesis of JIA and JIA-associated uveitis here, as this patient had total remission of both conditions with the onset of CVID.