5B00

ICD-11 MMS 2026-01

Autoimmune polyendocrinopathy

Description

This is a subtype of autoimmune polyendocrine syndrome, in which multiple endocrine glands dysfunction as a result of autoimmunity. It is a genetic disorder attributed to a defect in the AIRE gene that normally confers immune tolerance. It is inherited in a recessive fashion.

Index terms

  • Autoimmune polyendocrinopathy
  • Autoimmune polyglandular failure
  • Autoimmune polyglandular syndrome
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  • Lloyd syndrome
  • pluriglandular autoimmune atrophy
  • pluriglandular autoimmune syndrome
  • polyendocrine autoimmunity syndrome
  • polyglandular autoimmune deficiency
  • progressive pluriglandular insufficiency
  • thyroid-adrenocortical insufficiency syndrome
  • Autoimmune polyendocrinopathy type 1
  • APECED - [Autoimmune polyendocrinopathy - candidiasis - ectodermal dystrophy] syndrome
  • APS type 1- [autoimmune polyendocrine syndrome type 1]
  • Autoimmune hypoparathyroidism - chronic candidiasis - Addison's disease
  • Autoimmune polyendocrine syndrome type 1
  • Autoimmune polyendocrinopathy - candidiasis - ectodermal dystrophy syndrome
  • Autoimmune polyglandular syndrome type 1
  • HAM - [Hypoparathyroidism - Addison's disease - mucocutaneous candidiasis] syndrome
  • Hypoparathyroidism - Addison's disease - mucocutaneous candidiasis
  • MEDAC - [Multiple endocrine deficiency - Addison's disease - candidiasis] syndrome
  • Multiple endocrine deficiency - Addison's disease - candidiasis
  • Autoimmune polyendocrinopathy type 2
  • APS type 2- [autoimmune polyendocrine syndrome type 2]
  • Autoimmune polyendocrine syndrome type 2
  • Autoimmune polyglandular syndrome type 2
  • Autoimmune thyroid disease and/or type 1 diabetes - Addison disease
  • Schmidt syndrome
  • Autoimmune polyendocrinopathy type 3
  • APS type 3- [autoimmune polyendocrine syndrome type 3]
  • Autoimmune polyendocrine syndrome type 3
  • Autoimmune polyglandular syndrome type 3
  • Autoimmune polyendocrinopathy type 4
  • APS type 4- [autoimmune polyendocrine syndrome type 4]
  • Autoimmune polyendocrine syndrome type 4
  • Autoimmune polyglandular syndrome type 4

Exclusions from above levels

  • 4A01.31 – Ataxia-telangiectasia
  • 5A50.1 – Pseudohypoparathyroidism
  • 8C71.0 – dystrophia myotonica [Steinert]
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  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5B01 – Polyglandular hyperfunction
  • 5B0Y – Other specified polyglandular dysfunction
  • 5B0Z – Polyglandular dysfunction, unspecified

Foundation entity

Autoimmune polyendocrinopathy – http://id.who.int/icd/entity/548357900

Part of: 05: Endocrine, nutritional or metabolic diseases