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

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 -(Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes). 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.