Abstract
Autoimmune polyglandular syndrome (APS) is a rare disease with similar phenotypes but different, predominantly endocrine, organ involvement. The pathogenesis is uncertain. The autoimmune regulator gene (AIRE) on chromosome 21 has been identified for Type I, which also is associated with a predisposition to candidiasis. Type II is HLA related. Treatment of specific autoimmune diseases is crucial. Because these diseases are related to immunoglobulin G (IgG) autoantibodies, we describe 2 patients with APS for whom IVIg therapy was introduced 6–7 years ago to decrease the level of autoantibodies by increasing catabolism of IgG. There has been a salutary effect on some disease processes, specifically autoimmune hepatitis and production of adrenal autoantibodies.
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