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
|