Abstract

Polyglandular autoimmune syndrome (PAS) is a rare disease. It is 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). Our patient of PAS Type 2 with hypothyroidism and adrenal failure who achieved significant improvement after receiving cortisone & thyroid replacement therapy. Our patient is a 70 year old male, an atypical presentation, hence emphasizing that clinicians should have high index of suspicion for such condition. Mortality rate is high if patients are not diagnosed promptly and treated early. Patients with PAS require hormone replacement therapy life-long and monitoring for development of other autoimmune diseases.

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