Table 2.

Classification of autoimmune polyglandular syndromes (APS).

Category	Subtypes	Criteria
APS-1	—	Two or more from the following (i) Addison's disease (ii) Chronic candidiasis (iii) Hypoparathyroidism Associated conditions (i) Alopecia (ii) Autoimmune gastritis/pernicious anemia (iii) Type 1 diabetes (iv) Vitiligo (v) Autoimmune thyroid disease (vi) Chronic hepatitis (vii) Autoimmune-related gonadal failure
APS-2	—	Addison's disease plus any of the following (i) Type 1 diabetes (ii) Autoimmune thyroid disease
APS-3	APS-3A APS-3B APS-3C APS-3D	Autoimmune thyroid disease plus: type 1 diabetes with/without any other endocrine organ involvement Autoimmune thyroid disease plus: autoimmune gastrohepatic disease (inflammatory bowel syndrome, pernicious anemia, autoimmune gastritis, and primary biliary cirrhosis) Autoimmune thyroid disease plus: skin autoimmune disease (vitiligo with/without alopecia areata) with/without nervous system autoimmune disease (miastenia gravis, multiple sclerosis) Autoimmune thyroid disease plus: rheumatological autoimmune disease (systemic and discoid lupus, rheumatoid arthritis, Sjögren syndrome, systemic sclerosis, vasculitis, and antiphospholipid syndrome) with/without hematological disease
APS-4	—	Any other combination of specific organ and nonorgan specific autoimmune diseases