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

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Abstract

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.

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Authors and Affiliations

  1. Endocrine Unit, Department of Medicine (DIMED), University of Padua, Padua, Italy

    C. Betterle, C. Sabbadin & C. Scaroni

  2. Thornhill, Cardiff, UK

    J. Furmaniak

  3. Unit of Internal Medicine, Ospedale Dell’Angelo, Mestre-Venice, Italy

    F. Presotto

  4. Chair of Clinical Immunology and Allergy, Department of Medical and Surgical Sciences, University of Padua Medical School, Via Ospedale Civile 105, 35128, Padua, Italy

    C. Betterle

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Betterle, C., Furmaniak, J., Sabbadin, C. et al. Type 3 autoimmune polyglandular syndrome (APS-3) or type 3 multiple autoimmune syndrome (MAS-3): an expanding galaxy. J Endocrinol Invest 46, 643–665 (2023). https://doi.org/10.1007/s40618-022-01994-1

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