Autoimmunity and immunodeficiency disease.
There is a high incidence of autoimmune disease in patients with primary immunodeficiency. In almost all instances autoantibodies are to antigenic determinants of the formed elements of the blood--erythrocytes, platelets and neutrophils. In some rare cases, autoantibodies to T or B lymphocytes can cause immunodeficiency. IgA is unique in inciting autoantibodies to this serum protein in some patients with IgA deficiency. Organ-specific autoimmune disease is extremely rare in immunodeficiency states. In contrast, drug allergy and allergic contact dermatitis are extremely common in patients with deficiencies of B lymphocytes. Patients with the syndrome of immunodeficiency with elevated IgM (hyper-IgM deficiency) almost invariably develop autoantibodies to formed elements of the blood. Autoantibody production can be suppressed with anti-idiotypic antibodies.
Rosen
F S
FS
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115.
D000328:Adult; D000361:Agammaglobulinemia / Q000235:genetics; D001327:Autoimmune Diseases / Q000209:etiology* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007121:Immunocompetence; D007075:Immunoglobulin M / Q000276:immunology; D007153:Immunologic Deficiency Syndromes / Q000235:genetics / Q000276:immunology*; D066298:In Vitro Techniques; D007223:Infant; D008297:Male; D014960:X Chromosome
D007075:Immunoglobulin M