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Granulomatous Disease in Common Variable Immunodeficiency
- Laura J. Mechanic, MD;
- Steven Dikman, MD; and
- Charlotte Cunnigham-Rundles, MD, PhD
- From Mount Sinai Medical Center, New York, New York, Acknowledgments: The authors thank Drs. Bruce DeCottis, Barbara Chilmonczyk, Frank Church, Richard Daniels, Jerry Winklestein, Howard Lederman, Jeffrey Lobel, Alvin Tierstein, Kirk Sperber, Lloyd Mayer, and Scott Hennigan for referral of patients; Dr. Carol Bodian for statistical analyses; and Lydia Lopez for secretarial assistance. Grant Support: In part by grant CA 533H from the National Cancer Institute, grant NIH 5 M01-RR-0071 from the National Center for Research Resources for the Mount Sinai General Research Center, and grant FD-R-001162-01 from the Office of Orphan Products Development. Requests for Reprints: Charlotte Cunningham-Rundles, MD, PhD, Division of Clinical Immunology, Mount Sinai School Medical Center, 1452 Madison Avenue, New York, NY 10029. Current Author Addresses: Drs. Mechanic and Cunningham-Rundles: Division of Clinical Immunology, Mount Sinai School of Medicine, 1452 Madison Avenue, New York, NY 10029. Dr. Dikman: Division of Clinical Pathology, Mount Sinai School of Medicine, 1452 Madison Avenue, New York, NY 10029.
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.
- Copyright ©2004 by the American College of Physicians
