Autoimmune polyendocrine syndrome classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
On the basis of organ involvement, autoimmune polyendocrine syndrome (APS) can be classified into APS type 1, APS type 2 and APS type 3. APS type 1 commonly presents with mucocutaneous candidiasis, hypoparathyroidism and Addison's disease. APS type 2 commonly presents with Addison's disease, autoimmune thyroiditis and diabetes mellitus type 1. APS type 3 commonly 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.
| 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 (APS) type 3 can be further classified into APS type 3A, APS type 3B and APS type 3C.
| Autoimmune polyendocrine syndrome type 3 | |||||||||||||||||||||||||||||||||||
| 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 | |||||||||||||||||||||||||||||||||
Other diseases
Other diseases featuring polyendocrine autoimmunity:
- Chromosomal abnormalities (Down's syndrome) increase the risk of endocrine autoimmunity
- POEMS syndrome - the E is for endocrinopathy; the cause is a paraprotein excreted by a plasmacytoma or multiple myeloma; other features are polyneuropathy, organomegaly (hepatomegaly and splenomegaly), M-protein (paraprotein) and skin changes.