JUVENILE RHEUMATOID-ARTHRITIS AND COMMON VARIABLE HYPOGAMMAGLOBULINEMIA

We describe a 9-year-old white boy with systemic juvenile rheumatoid a rthritis (JRA) who developed pancytopenia and hypersplenism at the age of 13 years. He underwent splenectomy and 3 years later he developed Coombs' positive hemolytic anemia, alopecia, juvenile warts, and multi ple bacterial infections. At that time, investigations were compatible with severe hypogammaglobulinemia associated with common variable imm unodeficiency. Concomitantly with this condition he experienced comple te remission of his inflammatory arthritis. Immunologic studies of B a nd T lymphocyte function showed that the number of circulating T and B lymphocytes were normal, while T cell function was depressed, as evid enced by markedly reduced proliferative responses to mitogens and anti gens, and ability to mediate B cell help. In addition, his circulating B cells were unable to secrete IgM or IgG. He also exhibited anergy t o intradermal challenge with a battery of common antigens. The literat ure dealing with this clinical association is reviewed, and possible i mmunologic mechanisms involved are discussed.