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Condition

Autoimmune Polyglandular Syndrome Type 2

Condition 101

What is the definition of Autoimmune Polyglandular Syndrome Type 2?

Autoimmune polyglandular syndrome type 2 is an autoimmune disorder that affects many hormone-producing (endocrine) glands. It is characterized by the presence of Addison's disease along with autoimmune thyroid disease and/or type 1 diabetes. Affected individuals may also have p ...

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What are the alternative names for Autoimmune Polyglandular Syndrome Type 2?

  • Diabetes mellitus, Addison's disease, myxedema
  • Multiple endocrine deficiency syndrome, type 2
  • Polyglandular autoimmune syndrome, type 2
  • Autoimmune polyendocrine syndrome type 2
  • Schmidt syndrome
  • Schmidt's syndrome
  • APS2
  • PGA 2
  • Polyglandular deficiency syndrome type 2
  • Autoimmune polyglandular syndrome type II
  • PGA-II

What are the causes for 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. 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. In general, there is an  association with certain genes coding for proteins involved with the immune system, particularly of the major histocompatibility complex. For example, patients with APS-2 who are at risk for celiac disease generally have variants in DR3-DQ2 and DR4-DQ8, which also confer a risk of type 1 diabetes, autoimmune thyroid disease, and Addison’s disease.

Is Autoimmune Polyglandular Syndrome Type 2 an inherited disorder?

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. Genetic factors, perhaps related to HLA subtypes, appear to increase susceptibility to this condition. 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. There have been several reports of multiple family members with one or more endocrine deficiencies.

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Latest Research

Latest Advance
Study
  • Condition: Autoimmune Addison's Disease (AAD)
  • Journal: Best practice & research. Clinical endocrinology & metabolism
  • Treatment Used: Hydrocortisone (HC)
  • Number of Patients: 0
  • Published —
This article discusses autoimmune Addison's disease (AAD) and its treatments.
Latest Advance
Study
  • Condition: Genetic Hypoparathyroidism
  • Journal: Endocrine
  • Treatment Used: Teriparatide
  • Number of Patients: 6
  • Published —
This study tested the safety and efficacy of using teriparatide to treat patients aged a mean 9.4 years with genetic hypoparathyroidism.
Latest Advance
Study
  • Condition: Hypoparathyroidism
  • Journal: The Journal of pediatrics
  • Treatment Used: Parathyroid hormone injections
  • Number of Patients: 14
  • Published —
This study evaluated whether multiple daily injections of parathyroid hormone 1-34 are safe and effective as a long-term therapy for children with hypoparathyroidism.

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  • Start Date: November 5, 2012
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Clinical Trial
Other
  • Status: Active, not recruiting
  • Participants: 66
  • Start Date: July 30, 2009
Evaluation Genotypic, Phenotypic and Prognosis Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED) Syndrome