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5B00 Autoimmune polyendocrinopathy

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

code elsewhere

• X-linked immune dysregulation – polyendocrinopathy – enteropathy  (4A01.21)

synonyms

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

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