Granulomatous disease in common variable immunodeficiency

Abstract

Background: Granulomatous lesions are occasionally found in the lymphoid or solid organs of patients with common variable immunodeficiency.

Objective: To examine the clinical and immunologic conditions in patients with common variable immunodeficiency who have granulomas.

Design: Case series.

Setting: Large tertiary care medical center.

Patients: 17 hypogammaglobulinemic patients with common variable immunodeficiency whose organ or tissue biopsy samples contained noncaseating granulomas.

Measurements: Results of lymphocyte function tests.

Results: Eight of 17 patients had granulomas at some point before hypogammaglobulinemia was diagnosed. Sixteen of the 17 had deficient T-cell proliferation to mitogens. Although 14 patients received standard treatment with intravenous immunoglobulin, they have had substantial illness, including frequent autoimmune disease.

Conclusions: Dysregulated T-cell function or macrophage activation may have been involved in formation of granulomas and increased illness in hypogammaglobulinemic patients with common variable immunodeficiency. Delay in recognition of antibody deficiency may have contributed to the severity of illness in these patients.