Abstract
Autoimmune polyendocrine syndromes (APSs) are defined as a multiple autoimmune-mediated endocrine gland failure. The currently used classification criteria of APS, based on clinical data only, propose four main clinical presentations.
APS-1 is characterised by three main clinical features: chronic candidiasis, chronic hypoparathyroidism and Addison’s disease. APS-2 (or Schmidt syndrome) is a combination of Addison disease (AD), autoimmune thyroid disease (TAD) and/or Type 1 Diabetes Mellitus. APS-3 is defined as an association between TAD and another systemic or organ-specific autoimmune disease. Because TAD is the most prevalent autoimmune condition in the general population, APS-3 is the most frequently observed autoimmune polyendocrine syndrome. APS-4 includes other combinations of the autoimmune disorders previously described, which cannot be allocated in other APS definitions.
Most of the autoimmune endocrine diseases of APS are characterised and preceded by the development of organ-specific autoantibodies, usually directed to antigens expressed by endocrine cells. Different authors have suggested a common pathogenesis, basing on a similar mononuclear leukocyte infiltration and the detection of organ-specific autoantibodies.
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Tincani, A., Ceribelli, A., Cavazzana, I., Franceschini, F., Sulli, A., Cutolo, M. (2008). Autoimmune Polyendocrine Syndromes. In: Shoenfeld, Y., Cervera, R., Gershwin, M.E. (eds) Diagnostic Criteria in Autoimmune Diseases. Humana Press. https://doi.org/10.1007/978-1-60327-285-8_50
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DOI: https://doi.org/10.1007/978-1-60327-285-8_50
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