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Genetic and Rare Diseases Information Center (GARD)

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Autoimmune polyglandular syndrome type 1

Other Names for this Disease
  • APS 1
  • Autoimmune polyendocrine syndrome type 1
  • Autoimmune polyendocrinopathy syndrome type 1
  • Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)
  • Hypoadrenocorticism with hypoparathyroidism and superficial moniliasis
More Names
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How rare is autoimmune polyglandular syndrome type 1?

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What is autoimmune polyglandular syndrome type 1?

Autoimmune polyglandular syndrome type 1 is an inherited autoimmune condition that affects many of the body's organs. Symptoms often begin in childhood or adolescence and may include mucocutaneous candidiasis, hypoparathyroidism, and Addison disease. Affected individuals typically have at least two of these features, and many have all three. This syndrome can cause a variety of additional signs and symptoms, although they occur less often. Complications of this disorder can affect the skin and nails, the gonads (ovaries and testicles), the eyes, the thyroid, and the digestive system. Type 1 diabetes also occurs in some patients with this condition. Mutations in the AIRE gene cause autoimmune polyglandular syndrome, type 1. This condition is inherited in an autosomal recessive fashion.[1]
Last updated: 5/28/2009

How rare is autoimmune polyglandular syndrome type 1?

Autoimmune polyglandular syndrome type 1 is thought to be a rare condition, with about 500 cases reported worldwide. This condition occurs more frequently in certain populations, including Iranian Jews, Sardinians, and Finns.[1]
Last updated: 5/29/2009