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==Overview==
==Overview==
Autoimmune polyendocrine syndrome (APS) are a group of [[rare]] [[autoimmune]] [[disorders]]. APS type 2 is the most commonly seen autoimmune polyendocrine syndrome. The [[incidence]] of APS type 2 is estimated to be 1-2 per 100,000 individuals worldwide. The [[prevalence]] of APS type 2 is estimated to be 1-4 per 100,000 individuals worldwide. Most cases of APS type 1 and type 2 are [[symptomatic]] by early thirties, while APS type 3 is generally seen in 40-60 years of age. APS usually affects individuals of the Caucasian race. In APS type 1, type 2 and type 3 [[females]] are more commonly affected than [[men]].
Autoimmune polyendocrine syndrome (APS) are a group of [[rare]] [[autoimmune]] [[disorders]]. APS type 2 is the most commonly seen autoimmune polyendocrine syndrome. The [[incidence]] of APS type 2 is estimated to be 1-2 per 100,000 individuals worldwide. The [[prevalence]] of APS type 2 is estimated to be 1-4 per 100,000 individuals worldwide. Most cases of APS type 1 and type 2 are [[symptomatic]] by early thirties, while APS type 3 is generally seen in the fourth to sixth decade of life. APS usually affects individuals of the Caucasian race. In APS type 1, type 2 and type 3 [[females]] are more commonly affected than [[men]].


==Epidemiology and Demographics==
==Epidemiology and Demographics==
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===Incidence===
===Incidence===
*The [[incidence]] of autoimmune polyendocrine syndrome (APS) type 2 is estimated to be 1-2 per 100,000 individuals worldwide.  
*The [[incidence]] of autoimmune polyendocrine syndrome (APS) type 2 is estimated to be 1-2 per 100,000 individuals worldwide.  
*The most common autoimmune polyendocrine syndrome seen in general [[population]] is APS type 2.
*The most common autoimmune polyendocrine syndrome seen among the general [[population]] is APS type 2.
*APS type 1 and type 3 are very rare and extremely rare conditions respectively. Therefore no precise data is available for their [[incidence]].
*APS type 1 and type 3 are very rare and extremely rare conditions respectively. Therefore no precise data is available for their [[incidence]].


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===Age===
===Age===
*Autoimmune polyendocrine syndrome (APS) type 1 commonly affects [[children]] in the age group of 3-5 or in 13-15 years of [[age]].  
*Autoimmune polyendocrine syndrome (APS) type 1 commonly affects [[children]] of age group 3-5 years or in 13-15 years of [[age]].  
*Autoimmune polyendocrine syndrome (APS) type 2 commonly affects adults in the third and fourth decade of life.
*Autoimmune polyendocrine syndrome (APS) type 2 commonly affects adults in the third and fourth decade of life.
*Autoimmune polyendocrine syndrome (APS) type 3 commonly affects 40-60 years old [[women]] but may occur in any age group.
*Autoimmune polyendocrine syndrome (APS) type 3 commonly affects elderly [[women]] during fourth to sixth decades of life but may occur at any age.
*Most cases of autoimmune polyendocrine syndrome (APS) type 1 and type 2 are [[symptomatic]] by early thirties.
*Most cases of autoimmune polyendocrine syndrome (APS) type 1 and type 2 are [[symptomatic]] by early thirties.


Latest revision as of 21:08, 27 October 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Akshun Kalia M.B.B.S.[2]

Overview

Autoimmune polyendocrine syndrome (APS) are a group of rare autoimmune disorders. APS type 2 is the most commonly seen autoimmune polyendocrine syndrome. The incidence of APS type 2 is estimated to be 1-2 per 100,000 individuals worldwide. The prevalence of APS type 2 is estimated to be 1-4 per 100,000 individuals worldwide. Most cases of APS type 1 and type 2 are symptomatic by early thirties, while APS type 3 is generally seen in the fourth to sixth decade of life. APS usually affects individuals of the Caucasian race. In APS type 1, type 2 and type 3 females are more commonly affected than men.

Epidemiology and Demographics

The epidemiology and demographics of autoimmune polyendocrine syndrome (APS) is as follow:[1][2][3][4][5][6]

Incidence

  • The incidence of autoimmune polyendocrine syndrome (APS) type 2 is estimated to be 1-2 per 100,000 individuals worldwide.
  • The most common autoimmune polyendocrine syndrome seen among the general population is APS type 2.
  • APS type 1 and type 3 are very rare and extremely rare conditions respectively. Therefore no precise data is available for their incidence.

Prevalence

  • The prevalence of autoimmune polyendocrine syndrome (APS) type 2 is estimated to be 1-4 per 100,000 individuals worldwide.
  • APS type 1 and type 3 are very rare and extremely rare conditions respectively. Therefore no precise data is available for their prevalence.
  • APS type 1 is a very rare disorder and seen mostly in Iran, Sardinia and Finland.
    • The prevalence of APS type 1 is estimated to be 11 cases per 100,000 in Iranian Jews.
    • The prevalence of APS type 1 is estimated to be 7 cases per 100,000 in Sardinians.
    • The prevalence of APS type 1 is estimated to be 4 cases per 100,000 in Finland.

Age

  • Autoimmune polyendocrine syndrome (APS) type 1 commonly affects children of age group 3-5 years or in 13-15 years of age.
  • Autoimmune polyendocrine syndrome (APS) type 2 commonly affects adults in the third and fourth decade of life.
  • Autoimmune polyendocrine syndrome (APS) type 3 commonly affects elderly women during fourth to sixth decades of life but may occur at any age.
  • Most cases of autoimmune polyendocrine syndrome (APS) type 1 and type 2 are symptomatic by early thirties.

Race

  • Autoimmune polyendocrine syndrome (APS) usually affects individuals of the Caucasian race.

Gender

  • In autoimmune polyendocrine syndrome type 1, type 2 and type 3 females are more commonly affected than men.

Region

  • The majority of autoimmune polyendocrine syndrome type 1 cases are reported in Finland, Sardinia and Iran. It can be attributed to consanguineous marriages and/or clustering of descendants of common family founders. Other less frequent regions include Norway, Germany, northern Italy and northern Britain.

References

  1. ↑ Förster G, Krummenauer F, Kühn I, Beyer J, Kahaly G (1999). "[Polyglandular autoimmune syndrome type II: epidemiology and forms of manifestation]". Dtsch. Med. Wochenschr. (in German). 124 (49): 1476–81. doi:10.1055/s-2008-1035684. PMID 10629665.
  2. ↑ Neufeld M, Maclaren NK, Blizzard RM (1981). "Two types of autoimmune Addison's disease associated with different polyglandular autoimmune (PGA) syndromes". Medicine (Baltimore). 60 (5): 355–62. PMID 7024719.
  3. ↑ Heino M, Scott HS, Chen Q, Peterson P, Mäebpää U, Papasavvas MP, Mittaz L, Barras C, Rossier C, Chrousos GP, Stratakis CA, Nagamine K, Kudoh J, Shimizu N, Maclaren N, Antonarakis SE, Krohn K (1999). "Mutation analyses of North American APS-1 patients". Hum. Mutat. 13 (1): 69–74. doi:10.1002/(SICI)1098-1004(1999)13:1<69::AID-HUMU8>3.0.CO;2-6. PMID 9888391.
  4. ↑ Björses P, Halonen M, Palvimo JJ, Kolmer M, Aaltonen J, Ellonen P, Perheentupa J, Ulmanen I, Peltonen L (2000). "Mutations in the AIRE gene: effects on subcellular location and transactivation function of the autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy protein". Am. J. Hum. Genet. 66 (2): 378–92. doi:10.1086/302765. PMC 1288090. PMID 10677297.
  5. ↑ Rosatelli MC, Meloni A, Meloni A, Devoto M, Cao A, Scott HS, Peterson P, Heino M, Krohn KJ, Nagamine K, Kudoh J, Shimizu N, Antonarakis SE (1998). "A common mutation in Sardinian autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy patients". Hum. Genet. 103 (4): 428–34. PMID 9856486.
  6. ↑ Zlotogora J, Shapiro MS (1992). "Polyglandular autoimmune syndrome type I among Iranian Jews". J. Med. Genet. 29 (11): 824–6. PMC 1016181. PMID 1453436.

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