Type 3 autoimmune polyglandular syndrome (APS-3) or type 3 multiple autoimmune syndrome (MAS-3): an expanding galaxy

Background The number of recognised distinct autoimmune diseases (AIDs) has progressively increased over the years with more than 100 being reported today. The natural history of AIDs is characterized by progression from latent and subclinical to clinical stages and is associated with the presence o...

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Bibliographic Details
Published in:Journal of endocrinological investigation 2023-04, Vol.46 (4), p.643-665
Main Authors: Betterle, C., Furmaniak, J., Sabbadin, C., Scaroni, C., Presotto, F.
Format: Article
Language:English
Subjects:
Addison Disease
Autoantibodies
Autoimmune diseases
Autoimmunity
Case reports
Endocrinology
Hashimoto Disease
Humans
Internal Medicine
Medicine
Medicine & Public Health
Metabolic Diseases
Polyendocrinopathies, Autoimmune - diagnosis
Polyendocrinopathies, Autoimmune - epidemiology
Review
Syndrome
Thyroid diseases
Thyroid gland
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Summary:Background The number of recognised distinct autoimmune diseases (AIDs) has progressively increased over the years with more than 100 being reported today. The natural history of AIDs is characterized by progression from latent and subclinical to clinical stages and is associated with the presence of the specific circulating autoantibodies. Once presented, AIDs are generally chronic conditions. AIDs have the tendency to cluster and co-occur in a single patient. Autoimmune thyroid diseases (AITD) are the most prevalent of AIDs in the world population, and about one-third of the AITD patients also present with a non-thyroid AID during their life-span. Furthermore, patient with non-thyroid AIDs often presents with a form of AITD as a concurrent condition. Many of the clusters of AIDs are well characterized as distinctive syndromes, while some are infrequent and only described in case reports. Purpose In this review, we describe the wide spectrum of the combinations and the intricate relationships between AITD and the other AIDs, excluding Addison’s disease. These combinations are collectively termed type 3 Autoimmune Polyglandular Syndrome (APS-3), also called type 3 Multiple Autoimmune Syndrome (MAS-3), and represent the most frequent APS in the world populations. Conclusions Numerous associations of AITD with various AIDs could be viewed as if the other AIDs were gravitating like satellites around AITD located in the center of a progressively expanding galaxy of autoimmunity.
ISSN:0391-4097
1720-8386
DOI:10.1007/s40618-022-01994-1