Granulomatous disease in common variable immunodeficiency.

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.