title: 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.
  
  
  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.
authors.affiliation: Mount Sinai Medical Center, New York, New York, USA.
authors.forename: L J
authors.initials: LJ
authors.lastname: Mechanic
authors.forename: S
authors.initials: S
authors.lastname: Dikman
authors.forename: C
authors.initials: C
authors.lastname: Cunningham-Rundles
mesh_terms: 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
chemical_list: D007703:Peptidyl-Dipeptidase A