Polyglandular autoimmune syndromes

Dr Henry Knipe and Dr Jeremy Jones et al.

Polyglandular autoimmune syndromes (PAS) are a rare set of diseases characterised by the presence of ≥2 autoimmune endocrine disease.

Pathology

Three types of PAS have been described. 

PAS type I
  • a.k.a. APECED (autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy) or MEDAC (multiple endocrine deficiency autoimmune candidiasis syndrome) 2
  • onset: 3-5 years 2
  • chronic mucocutaneous candidiasis
  • acquired hypoparathyroidism
  • Addison disease
PAS type II
PAS type III
  • absence of Addison disease otherwise identical to PAS type III 2,3
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Article Information

rID: 1905
Section: Syndromes
Synonyms or Alternate Spellings:
  • Polyglandular autoimmune syndromes (PAS)
  • APECED (autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy)
  • MEDAC (multiple endocrine deficiency autoimmune candidiasis syndrome)

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