Autoimmune polyendocrine syndrome classification

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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) can be classified into APS type 1, APS type 2 and APS type 3 on the basis of organ involvement. APS type 1 commonly presents with mucocutaneous candidiasis, hypoparathyroidism and Addison's disease. APS type 2 most commonly presents with Addison's disease, autoimmune thyroiditis and diabetes mellitus type 1. APS type 3 usually presents with autoimmune thyroiditis, diabetes mellitus type 1 and pernicious anemia.

Classification

On the basis of organ involvement, autoimmune polyendocrine syndrome (APS) can be classified into APS type 1, APS type 2 and APS type 3.[1][2][3]


 
 
 
 
 
 
 
 
Autoimmune polyendocrine syndrome (APS)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Autoimmune polyendocrine syndrome type 1
 
 
 
 
Autoimmune polyendocrine syndrome type 2
 
 
 
 
Autoimmune polyendocrine syndrome type 3
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
•Mucocutaneous candidiasis
Hypoparathyroidism
Addison's disease
 
 
 
 
Addison's disease
Autoimmune thyroiditis
Diabetes mellitus type 1
 
 
 
 
Autoimmune thyroiditis
Diabetes mellitus type 1
Pernicious anemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Autoimmune polyendocrine syndrome type 3A
 
 
 
Autoimmune polyendocrine syndrome type 3B
 
 
 
 
 
Autoimmune polyendocrine syndrome type 3C
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Autoimmune thyroiditis
•Immune mediated diabetes mellitus
 
 
 
Autoimmune thyroiditis
Pernicious anemia
 
 
 
 
 
Autoimmune thyroiditis
Vitiligo/Alopecia

References

  1. Betterle C, Zanchetta R (2003). "Update on autoimmune polyendocrine syndromes (APS)". Acta Biomed. 74 (1): 9–33. PMID 12817789.
  2. Forcier RJ, McIntyre OR, Frey WG, Andrada JA, Streiff RR (1972). "Autoimmunity and multiple endocrine abnormalities". Arch. Intern. Med. 129 (4): 638–41. PMID 5067225.
  3. Eisenbarth GS, Gottlieb PA (2004). "Autoimmune polyendocrine syndromes". N. Engl. J. Med. 350 (20): 2068–79. doi:10.1056/NEJMra030158. PMID 15141045.

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