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.


METHODS
Case series.


METHODS
Large tertiary care medical center.


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


METHODS
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.
Mechanic
L J
LJ
Mount Sinai Medical Center, New York, New York, USA.
Dikman
S
S
Cunningham-Rundles
C
C
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000276:immunology / Q000473:pathology; D005260:Female; D006099:Granuloma / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006801:Humans; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D007703:Peptidyl-Dipeptidase A / Q000097:blood; D012507:Sarcoidosis / Q000150:complications / Q000175:diagnosis; D013601:T-Lymphocytes / Q000276:immunology
D007703:Peptidyl-Dipeptidase A