AUTOIMMUNITY IN RELATIVES OF PATIENTS WITH IMMUNODEFICIENCY DISEASES

  • 1 January 1977
    • journal article
    • research article
    • Vol. 28  (3) , 375-388
Abstract
Autoimmune disorders are reportedly more frequent than expected in immunodeficient patients and in their relatives. The hypothesis that genetic factors related to immunodeficiency may predispose to the development of autoimmunity was studied in relatives of patients with variable immunodeficiency (VID), ataxia telangiectasia (A-T) or X-linked infantile agammaglobulinemia (X-LA). Close relatives of patients with VID or A-T had thyroid and gastric autoantibodies significantly more frequently than did control subjects. No abnormalities were detected in unaffected relatives of X-LA patients. The increased incidence of organ-specific autoantibodies in close relatives of VID patients was confined to those families with more than 1 member with immunodeficiency. There apparently are at least 2 forms of VID, one of which is associated with familial autoimmunity. Heterozygous carriers of the A-T gene and persons with genes involved in the development of VID may exhibit T[thymus-derived]-lymphocyte dysfunction which predisposes them to autoimmunity.