Polyglandular Autoimmune Syndromes

Polyglandular autoimmune syndrome (PAS) is a rare disease. It characterized by the association of two or more organ-specific autoimmune disorders. It is estimated that about one-fourth of patients with a single organ-specific autoimmune disease may have or develop other autoimmune diseases. The syndrome can be classified into two types, type I and type II, each with distinct clinical characteristics. PAS type II is the coexistence of adrenal failure with autoimmune thyroid disease, or Type 1 diabetes mellitus (DM). We present a 43-year-old female with Type 1 DM, Hashimoto's thyroiditis and adrenal failure who achieved significant improve-ment after receiving cortisone replacement therapy. Her human leukocyte antigen typing showed DR 0309 and 0901. Patients with PAS require hormone replacement therapy and monitoring for development of other autoimmune diseases. (Tzu Chi Med J 2006