Table 1. Classification of autoimmune polyglandular syndrome [1-18]

Category

Clinical features

Criteria

APS-1

 

Addison disease, Chronic candidiasis, hypoparthyroidism

 

At least two of the three conditionsmust be present

Associated immune diseases (alopecia, pernicious anemia, gonadal failure, chronic active hepatitis, AITD, vitiligo, type 1 diabetes mellitus)

 

APS-2

 

Addison disease, AITD, type 1 diabetes mellitus

 

Addison disease + this/these associated disease

Associated immune disorders

 

APS-3

 

AITD, with one of the autoimmune diseases

 

AITD without Addison disease/ hypoparathyroidism, associated one of organ specific autoimmune disease listed in A;B;C;D

 

APS-3A

 

Type I diabetes mellitus with/without other endocrine disease ( gonadal failure)

 

AITD+ Autoimmune endocrine disease

 

APS-3B

 

Gastrointestinal or hepatic autoimmune diseases (perncious anemia,inflammatory bowel disease, auitoimmune hepatitis, autoimmune gastritis, primary biliary cirrhosis)

 

AITD+ Autoimmune gastrohepatic disease

 

APS-3C

 

Vitiligo with/without alopecia areata and/ or myasthenia gravis, multiple sclerosis

 

AITD+ skin ± neuromuscular-nervous system autoimmune disease

 

APS-3D

 

Systemic and discoid lupus erythematosus, rheumatoid arthritis, seronegative arthritis, systemic sclerosis, Sjögren syndrome, Werlhof syndrome, antiphospholipid syndrome, vasculitis)

 

AITD+ rheumatological autoimmune disease ± hematologic disease

 

APS-4

Other autoimmune disease not included previous groups

Different combinations with/without non-organ spesific autoimmune disease