[Autoimmune disease in primary antibody deficiencies].

BACKGROUND: Immunodeficiency and autoimmune phenomena may occur concomitantly in the same individual. Many immune deficiency syndromes, mainly humoral defects, are associated with autoimmune disorders. In the present study, we describe the frequency of autoimmune disorders in these patients.

METHODS AND RESULTS: We performed a retrospective review of the medical records of a cohort of 152 patients with primary antibody deficiency in the Clinical Immunology Unit of the Gregorio Maranon University Hospital in Madrid: common variable immunodeficiency (CVID) (n = 35), IgA deficiency (n = 43), specific IgG subclass deficiency (n = 56), antibody deficiency with normal immunoglobulin (n = 14) and X-linked agammaglobulinemia (n = 4). Of the 152 patients with primary antibody deficiencies, autoimmune disorders were found in 35 (23 %). Autoimmune hematologic disease was found in 14 patients (9.2 %), autoimmune diseases of the gastrointestinal tract in eight (5.3 %), autoimmune endocrine diseases in six (3.9 %), autoimmune rheumatic diseases in six (3.9 %) and vitiligo in four. Autoimmune diseases were more frequent in CVID patients (37.1 %) than in those with selective IgA deficiency (25.5 %) or selective IgG subclass deficiency (12.5 %). Thirty-seven percent of autoimmune episodes occurred prior to the diagnosis of primary antibody deficiency. Sixteen patients (10.5 %) had autoantibodies without clinical data of an autoimmune disease.

CONCLUSIONS: Primary antibody deficiencies have variable autoimmune manifestations. For early detection and appropriate treatment, autoimmune disease should be suspected in patients with immunodeficiency.