Adrenal gland and paraganglia
Adrenal insufficiency
Polyglandular autoimmune syndromes

Reviewer: Nat Pernick, M.D. (see Authors page)

Revised: 1 March 2016, last major update February 2005

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PubMed Search: Polyglandular autoimmune syndromes
Table of Contents
Definition / General
Definition / General
  • More common in women

    Type I polyglandular autoimmune syndrome:
    • Also called autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)
    • Due to mutations in gene at 21q22.3
    • Rare with < 200 cases reported
    • Autosomal recessive; associated with adrenal insufficiency in 60% of cases, usually by age 13 years
    • Also chronic mucocutaneous candidiasis and hypoparathyroidism in childhood
    • Also autoimmune thyroiditis, diabetes mellitus; variable alopecia; most have autoantibodies to glutamic acid decarboxylase

    Type II polyglandular autoimmune syndrome:
    • Usually autosomal dominant
    • Adrenal insufficiency in all cases; more common than type I
    • 50% familial with onset between ages 20-40 years
    • Also autoimmune thyroiditis (Grave disease), insulin dependent diabetes mellitus
    • Also primary hypogonadism, myasthenia gravis and celiac disease; only rarely hypoparathyroidism