Autoimmune polyglandular failure

This code represents a rare group of disorders characterized by the simultaneous or sequential failure of multiple endocrine glands due to an autoimmune process. It typically involves at least two endocrine glands, such as the adrenal glands, thyroid, parathyroid, or pancreas. The specific constellation of affected glands can vary, leading to diverse clinical presentations.

Use this code when documentation clearly indicates a diagnosis of autoimmune polyglandular syndrome (APS), also known as polyglandular autoimmune syndrome (PAS), or Schmidt's syndrome. This diagnosis is supported by evidence of autoimmune destruction and hypofunction of multiple endocrine organs.

• Always code the specific endocrine disorders contributing to the polyglandular failure in addition to this code.
• Distinguish from isolated autoimmune endocrinopathies; this code requires involvement of multiple glands.
• Review documentation for specific types of APS (e.g., Type 1, Type 2, Type 3) if specified, as these may have additional associated codes.