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Autoimmune polyendocrinopathy

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Disease definition

A group of rare endocrine diseases characterized by autoimmune activity against more than one endocrine organ, with possible additional involvement of non-endocrine organs. Autoimmunity is typically directed against different target antigens in different tissues. The two more common autoimmune polyendocrine syndromes (APS), APS type 1 and type 2, have a strong genetic background and have Addison's disease as a major feature. The group furthermore includes APS type 3 and type 4.

ORPHA:282196

Classification level: Group of disorders

Synonym(s):
  • APS
  • Autoimmune polyglandular syndrome

Prevalence: -

Inheritance: -

Age of onset:

ICD-10: E31.0

ICD-11: 5B00

UMLS: C0085409

MeSH: D016884

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2022.pdf) - Orphanet Urgences
Polski (2010.pdf) - Orphanet Urgences
Deutsch (2014.pdf) - Orphanet Urgences
English (2010.pdf) - Orphanet Urgences
Español (2010.pdf) - Orphanet Urgences
Português (2010.pdf) - Orphanet Urgences
Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Newborn screening

The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.