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Genetic and Rare Diseases Information Center (GARD)

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Autoimmune polyglandular syndrome type 2

Other Names for this Disease
  • APS2
  • Autoimmune polyendocrine syndrome type 2
  • Diabetes mellitus, Addison's disease, myxedema
  • Multiple endocrine deficiency syndrome, type 2
  • PGA 2
More Names
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Your Question

What causes autoimmune polyglandular syndrome type 2? Is it inherited?

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What is autoimmune polyglandular syndrome type 2?

Autoimmune polyglandular syndrome type 2 is an autoimmune disorder that affects many hormone-producing (endocrine) glands.[1] This condition is characterized by the presence of Addison's disease along with autoimmune thyroid disease and/or type 1 diabetes.[1] Affected individuals may also have problems with other endocrine glands. Autoimmune polyglandular syndrome type 2 is diagnosed in adulthood, typically around age 30.[2] This condition occurs more often in women than men, and treatment primarily involves hormone replacement therapy.[1] The cause of autoimmune polyglandular syndrome type 2 is unknown, although it may involve a combination of genetic and environmental factors.[1]
Last updated: 3/24/2010

What causes autoimmune polyglandular syndrome type 2?

While the cause of polyglandular autoimmune syndrome type 2 is not fully understood, it usually results from an autoimmune reaction, probably triggered by a virus or other environmental antigen.[1][2] There is some evidence of an association between diabetes or hypothyroidism and congenital rubella infection. Other cases may result from immune stimulation caused by certain dietary proteins. Genetic susceptibility and idiopathic immunopathologic dysfunction may also play a role.[1]
Last updated: 3/24/2010

Is autoimmune polyglandular syndrome type 2 inherited?

Although most cases of autoimmune polyglandular syndrome type 2 are sporadic, some clinical researchers believe that there is a familial or hereditary trait associated with it.[1][3][4] Genetic factors, perhaps related to HLA subtypes, appear to increase susceptibility to this condition.[2] In addition, several different types of inheritance (autosomal recessive, autosomal dominant, multifactorial) have been reported, suggesting that it may involve a complex interaction among many genes.[2][3][5] There have been several reports of multiple family members with one or more endocrine deficiencies.[4]
Last updated: 3/24/2010