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Mucocutaneous candidiasis and autoimmunity against cytokines in APECED and thymoma patients: clinic and pathogenetic implications

Lookup NU author(s): Dr Desa Lilic

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Abstract

Much has been learnt about the mechanisms of thymic self-tolerance induction from work on both the rare autosomal recessive disease autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) and the autoimmune regulator (AIRE) protein mutated in this disease. Normally, AIRE drives low-level expression of huge numbers of peripheral tissue-specific antigens (TSAgs) in medullary thymic epithelial cells (mTECs), leading to the deletion of TSAg-reactive thymocytes maturing nearby. The very recently discovered neutralizing autoantibodies (autoAbs) against Th17-related cells and cytokines in two autoimmunity-related syndromes associated with AIRE-mutant thymi or AIREdeficient thymomas help to explain the chronic mucocutaneous candidiasis (CMC) seen in both syndromes. The surprising parallels between these syndromes also demand new hypotheses and research into the consequences of AIRE deficiency and the ensuing autoimmunizing pathways, and suggest more appropriate treatment regimens as discussed in this review.

Publication metadata

Author(s): Lilic D; Kisand K; Casanova J-L; Peterson P; Meager A; Willcox N

Publication type: Review

Publication status: Published

Journal: European Journal of Immunology

Year: 2011

Volume: 41

Issue: 6

Pages: 1517-1527

Print publication date: 13/05/2011

ISSN (print): 0014-2980

ISSN (electronic): 1521-4141

URL: http://dx.doi.org/10.1002/eji.201041253

DOI: 10.1002/eji.201041253

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