E31.0

Billable

Autoimmune polyglandular failure

HCC Category Mapping

V28HCC 51 — Other Significant Endocrine and Metabolic Disorders

0.233

V24HCC 23 — Other Significant Endocrine and Metabolic Disorders

0.230

ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders

0.000

RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders

0.000

What This Code Means

A condition where the immune system attacks multiple glands in the body, causing them to fail and stop producing necessary hormones.

Coding Tips

•Document which specific glands are affected (thyroid, adrenal, parathyroid, etc.) for more specific coding if available
•This is often associated with Addison's disease and hypothyroidism; review documentation for additional diagnoses to code

Clinical Significance

Autoimmune polyglandular failure (autoimmune polyendocrine syndrome) involves immune-mediated destruction of multiple endocrine glands simultaneously, most commonly affecting adrenal, thyroid, and pancreatic islet cells. Type I (autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy) presents in childhood, while Type II (Schmidt syndrome) is more common and presents in adulthood with Addison disease, autoimmune thyroid disease, and type 1 diabetes mellitus.

Documentation Requirements

✓Document which specific glands are affected, autoimmune antibody panel results, individual hormone deficiencies and replacement therapies, genetic testing if applicable (AIRE gene for Type I), associated non-endocrine autoimmune conditions, and comprehensive management plan.

Commonly Confused Codes

E31.8 (other polyglandular dysfunction without autoimmune etiology), E31.1 (polyglandular hyperfunction rather than failure), E27.1 (primary adrenocortical insufficiency as a single-gland diagnosis), E06.3 (autoimmune thyroiditis as a single-gland diagnosis).

Code Hierarchy

└E31Polyglandular dysfunction └E31.0Autoimmune polyglandular failure

└E31.0Autoimmune polyglandular failure