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Journal of Clinical Immunology

, Volume 35, Issue 5, pp 463–478 | Cite as

Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy

  • Kai Kisand
  • Pärt Peterson
CME Review

Abstract

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is an autosomal recessive disease caused by mutations in the autoimmune regulator (AIRE) gene. This review focuses on the clinical and immunological features of APECED, summarizes the current knowledge on the function of AIRE and discusses the importance of autoantibodies in disease diagnosis and prognosis. Additionally, we review the outcome of recent immunomodulatory treatments in APECED patients.

Keywords

APECED APS1 AIRE chronic mucocutaneous candidiasis type I interferons IL-17 IL-22 autoantibodies 

Abbreviations

AD

Addison’s disease

AIH

Autoimmune hepatitis

APECED

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy

APS1

Autoimmune polyendocrine syndrome, type 1

CMC

Chronic mucocutaneous candidiasis

EE

Enteroendocrine cells

ELISA

Enzyme-linked immunosorbent assay

GID

Gastrointestinal dysfunction

HP

Hypopathyroidism

IFN

Interferon

IL

Interleukin

ILD

Interstitial lung disease

LIPS

Luciferase based immunoprecipitation system

mTEC

Thymic medullary epithelial cell

T1D

Type 1 diabetes

TIN

Tubulo-interstitial nephritis

TSA

Tissue-specific antigens

Notes

Acknowledgments

This work was supported by Estonian Research Council grant IUT2-2, Center of Excellence of Translational Medicine of University of Tartu, European Regional Development Fund, and Archimedes Foundation.

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© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Institute of Biomedicine and Translational MedicineUniversity of TartuTartuEstonia

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