" /> Autoimmune polyendocrinopathy - CISMeF





ICD-11 code : 5B00;

Preferred Label : Autoimmune polyendocrinopathy;

ICD-11 definition : This 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.;

ICD-11 synonym : polyendocrine autoimmunity syndrome; Autoimmune polyglandular failure; Lloyd syndrome; Autoimmune polyglandular syndrome; polyglandular autoimmune deficiency;

ICD-11 inclusion : progressive pluriglandular insufficiency; thyroid-adrenocortical insufficiency syndrome; pluriglandular autoimmune atrophy; pluriglandular autoimmune syndrome;

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This 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.

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18/05/2024


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