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Adrenal gland and paraganglia

Adrenal insufficiency

Polyglandular autoimmune syndromes


Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 6 February 2013, last major update February 2005
Copyright: (c) 2002-2013, PathologyOutlines.com, Inc.

General
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● 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’s disease), insulin dependent diabetes mellitus
● Also primary hypogonadism, myasthenia gravis and celiac disease; only rarely hypoparathyroidism

End of Adrenal gland and paraganglia > Adrenal insufficiency > Polyglandular autoimmune syndromes


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