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Polyglandular Autoimmune Syndrome

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  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 19 Accesses

Synonyms

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy; Autoimmune polyglandular syndrome; Polyglandular autoimmune syndrome

Definition

A group of genetic autoimmune diseases characterized by autoimmune inflammation in endocrine tissues and other tissues that result in the following endocrine clinical manifestations: hypoparathyroidism, hypogonadism, adrenal insufficiency, insulin-dependent (type 1) diabetes mellitus, and hypothyroidism. Non-endocrine features include alopecia totalis, vitligo, inflammation of the cornea and keratoconjunctivitis, tooth enamel hypoplasia, pernicious anemia, malabsoprtion, hepatitis, and candidiasis. There are three types: Type 1 features hypoparathyroidism, adrenal insufficiency, hypogonadism, vitiligo, and candidiasis. Type 2, previously known as Schmidt syndrome (Schmidt 1926) features adrenal insufficiency, hypothyroidism, and diabetes mellitus. Type 3, also known as Immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX)...

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References and Further Reading

  • Husebye, E. S., Anderson, M. S., & Kampe, O. (2018). Autoimmune polyendocrine syndromes. The New England Journal of Medicine, 378, 1132–1141.

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  • Kahaly, G. J., & Frommer, L. (2018). Polyglandular autoimmune syndromes. Journal of Endocrinological Investigation, 41, 91–98.

    Article  CAS  Google Scholar 

  • Peterson, P., & Peltonen, L. (2005). Autoimmune polyendocrinopathy syndrome type 1 (APS1) and AIRE gene: New views on molecular basis of autoimmunity. Journal of Autoimmunity, 25(Suppl), 49–55.

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  • Schmidt, M. B. (1926). Eine biglandulare Erkrankung (Nebennieren und Schilddrüse) bei Morbus Adisonii. Verhandlungen der Deutschen Gesellschaft für Pathologie, 21, 212–221.

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Author information

Authors and Affiliations

  1. University Hospitals, Cleveland Medical Center, Case Western Reserve University, Cleveland, OH, USA

    Sylvia L. Asa

Authors
  1. Sylvia L. Asa
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Correspondence to Sylvia L. Asa .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Asa, S.L. (2020). Polyglandular Autoimmune Syndrome. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5052-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5052-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

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