Granulomatous disease in common variable immunodeficiency.
Granulomatous disease occurs in 8-22% of patients with common variable immunodeficiency (CVID). We examined the clinical and immunologic information of all 37 of 455 (8.1%) CVID subjects with this complication. The median age at diagnosis of CVID was 26 (2-59). 14 had granulomas 1-18 years before diagnosis of CVID. In 6 detection of granulomas coincided with this diagnosis; for 17, granulomas were documented later. 54% had lung granulomas, 43% in lymph nodes and 32% in liver. 54% of the group had had autoimmune diseases, mostly immune thrombocytopenia and hemolytic anemia. 24% had had a splenectomy. Nineteen (51.3%) required steroid treatment for granulomas; other immune suppressants were used in some. Over 25 years 28.5% died (median age 37.5), but not significantly more when compared to our CVID patients without granulomas (19.8%). Those with lung granulomas had similar mortality to those with granulomas in other tissues.
Ardeniz
Omür
O
Ege University Medical Faculty, Department of Internal Medicine, Division of Allergy and Clinical Immunology, Izmir, Turkey.
Cunningham-Rundles
Charlotte
C
D000293:Adolescent; D000328:Adult; D000906:Antibodies / Q000097:blood / Q000276:immunology; D001327:Autoimmune Diseases / Q000209:etiology / Q000276:immunology / Q000628:therapy; D001402:B-Lymphocytes / Q000166:cytology / Q000276:immunology; D016516:CD4-CD8 Ratio; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006099:Granuloma / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000401:mortality / Q000473:pathology; D006801:Humans; D008168:Lung / Q000473:pathology; D008213:Lymphocyte Activation / Q000187:drug effects / Q000276:immunology; D008297:Male; D008875:Middle Aged; D008934:Mitogens / Q000494:pharmacology; D013156:Splenectomy; D013601:T-Lymphocytes / Q000187:drug effects / Q000276:immunology; D055815:Young Adult
D000906:Antibodies; D008934:Mitogens