Arthritis associated with primary agammaglobulinemia: new clues to its immunopathology

Clin Exp Rheumatol. 1993 Jan-Feb;11(1):65-9.

Abstract

Primary agammaglobulinemia is a rare disorder which is associated with articular symptoms in 11% of patients. Septic arthritis may occur, but often patients complain of chronic oligoarthritis and have a clinical presentation similar to rheumatoid arthritis. We report 2 cases of primary agammaglobulinemia in adults, associated with non-erosive chronic arthritis. Peripheral blood lymphocyte phenotyping showed a predominance of CD8 lymphocytes with a CD4/CD8 ratio < 1. We did not find any abnormalities in cellular immunity. A histological study of the synovium showed chronic synovitis with perivascular CD8 lymphocyte infiltrates. Intravenous infusion of immunoglobulins resulted in a dramatic improvement in the arthritis in both cases. In one patient we noticed a decrease in CD8 lymphocytosis. These results suggest that CD8 lymphocytes are involved in the pathogenesis of the arthritis associated with agammaglobulinemia.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Agammaglobulinemia / complications*
  • Agammaglobulinemia / immunology*
  • Agammaglobulinemia / pathology
  • Arthritis / complications*
  • Arthritis / immunology*
  • Arthritis / pathology
  • CD8 Antigens / analysis
  • Chronic Disease
  • Female
  • Humans
  • Lymphocytes / immunology
  • Lymphocytes / pathology
  • Lymphocytes / physiology
  • Male
  • Synovial Membrane / pathology

Substances

  • CD8 Antigens