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Autoimmune polyendocrinopathy type 4

Disease definition

A rare autoimmune polyendocrinopathy characterized by autoimmune activity against an endocrine organ in combination with at least one more endocrine or non-endocrine organ. Typical autoimmune diseases occurring in this type include insulin-requiring diabetes, pernicious anemia, alopecia, vitiligo, or myasthenia gravis, but not Addison disease, thyroid disease, or hypoparathyroidism.

ORPHA:227990

Classification level: Disorder
  • Synonym(s):
    • APS type 4
    • APS4
    • Autoimmune polyendocrine syndrome type 4
    • Autoimmune polyglandular syndrome type 4
  • Prevalence: -
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: Adult
  • ICD-10: E31.0
  • ICD-11: 5B00
  • OMIM: -
  • UMLS: C3266026
  • MeSH: -
  • GARD: -
  • MedDRA: -

  A summary on this disease is available in Español (2020) Français (2020) Nederlands (2020)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site 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.