Autoimmune Polyglandular Syndrome

Yesterday we talked about an interesting case of a young male with a history of Type I DM and Celiac disease who presented with severe hyperkalemia (8mmol/L), hyponatremia, hypotension and non specific abdominal complaints.

With that degree of hyperkalemia and his history of multiple autoimmune diseases, the working diagnosis was primary adrenal insufficiency and possibly Polyglandular Autoimmune Syndrome type II.

1) Autoimmune Polyglandular Syndrome type I (AKA Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy APECED)

• Hypoparathyroidism
• Chronic mucocutaneous candidiasis
• Primary adrenal insufficiency (Addison's disease)
• hypogonadism
• Malabsorption and other gastrointestinal disorders

2) Polyglandular Autoimmune Syndrome type II (much more common)

• Type I DM
• Autoimmune thyroiditis, occasionally Graves disease
• Primary adrenal insufficiency
• Secondary adrenal insufficiency from autoimmune hypophysitis
• Hypogonadism
• Non-endocrine autoimmune phenomenon (vitiligo, myasthenia gravis, TTP)
• Schmidt's syndrome: primary adrenal insufficiency and autoimmune thyroid disease