Polyglandular autoimmune syndromes (PAS) are a rare set of diseases characterized 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
- a.k.a. Schmidt or Carpenter syndrome 3
- onset: 20-60 years 2
- Addison disease
- type 1 diabetes mellitus
- autoimmune thyroiditis
- hypoparathyroidism (rare)
- hypopituitarism (rare)
PAS type III
- absence of Addison disease otherwise identical to PAS type III 2,3