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.