Autoimmune polyendocrine syndrome epidemiology and demographics

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

Epidemiology and Demographics

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 in 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 is estimated to be 11 cases per 100,000 in Iranian Jews.
  • The prevalence of APS type is estimated to be 7 cases per 100,000 in Sardinians.
  • The prevalence of APS type is estimated to be 4 cases per 100,000 in Finland.

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 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.
• 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.