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Autoimmune Polyendocrine Syndromes (APS) or Multiple Autoimmune Syndromes (MAS)

An Overview

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Polyendocrine Disorders and Endocrine Neoplastic Syndromes

Part of the book series: Endocrinology ((ENDOCR))

Abstract

On the basis of the revised criteria of diagnosis of autoimmune diseases (AID), more than 80 diseases previously considered as “idiopathic” have been defined as “autoimmune” and estimated to affect about 7% of the general population. AID are classically divided into organ-specific and non-organ-specific, and their prevalence varies according to gender, geographical origin, age, and genetic predisposition. AID shows the tendency to aggregate in one individual or in a family, defining an autoimmune polyendocrine syndrome (APS), also called multiple autoimmune syndrome (MAS).

In this chapter, we revised the main features of the four types of APS/MAS, of IPEX and POEMS, evaluating their history, epidemiology, genetic pattern, clinical and immunological features, management, and therapy.

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Acknowledgments

This study was supported in part by a grant from the EU Seventh Framework Programme, Euradrenal project: Pathophysiology and Natural Course of Autoimmune Adrenal Failure in Europe. Grant No. 2008-201167, and by a grant ex 60% from the University of Padua.

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Authors and Affiliations

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

    Corrado Betterle, Chiara Sabbadin & Carla Scaroni

  2. Unit of Internal Medicine, Ospedale dell’Angelo, Mestre-Venezia, Italy

    Fabio Presotto

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  1. Corrado Betterle
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  2. Chiara Sabbadin
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  3. Carla Scaroni
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  4. Fabio Presotto
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Correspondence to Chiara Sabbadin .

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Editors and Affiliations

  1. Department of Clinical Medicine and Surgery, Section of Endocrinology - Neuroendocrine Unit, University of Naples “Federico II”, Naples, Italy

    Annamaria Colao

  2. Department of Biotechnological and Applied Clinical Sciences, University of L’Aquila, L’Aquila, Italy

    Marie-Lise Jaffrain-Rea

  3. Department of Endocrinology, University of Liège, Centre Hospitalier, Universitaire de Liège, Liège, Belgium

    Albert Beckers

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Betterle, C., Sabbadin, C., Scaroni, C., Presotto, F. (2021). Autoimmune Polyendocrine Syndromes (APS) or Multiple Autoimmune Syndromes (MAS). In: Colao, A., Jaffrain-Rea, ML., Beckers, A. (eds) Polyendocrine Disorders and Endocrine Neoplastic Syndromes. Endocrinology. Springer, Cham. https://doi.org/10.1007/978-3-319-89497-3_1

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