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New insights into the pathogenesis of Behçet's disease.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Pineton de Chambrun, Marc Wechsler, Bertrand Geri, Guillaume Cacoub, Patrice Saadoun, David
Description	Country affiliation: France Author Affiliation: Pineton de Chambrun M ( Department of Internal Medicine and Laboratory I3 Immunology, Immunopathology, Immunotherapy, UMR CNRS 7211, INSERM U959, Groupe Hospitalier La Pitié-Salpetrière, Université Pierre et Marie Curie, Paris 6, Paris, France)
Abstract	Behçet's disease (BD) is a recurrent systemic inflammatory disorder of unknown origin characterized by oral and genital mucous ulcer, uveitis, and skin lesions. Involvement of large vessels, central nervous system (CNS), gastrointestinal tract and thrombotic events are less frequent but can be life threatening. The aim of this review is to provide new insights into the pathogenesis of BD. Over the past year substantial advances have been done in the understanding of the genetic [1,2] and immunology [3] of BD. BD is at the crossroad between autoimmune and autoinflammatory syndromes. In common with autoimmune diseases BD shares class I MHC association. However, in contrast to autoimmune disorders, BD has clinical features that seem to be mostly autoinflammatory. The pathogenesis of BD is still unknown, but major determinants of the genetic and immune system abnormalities have been reported recently. Triggering infectious factors are supposed to participate in the outbreak of BD in genetically predisposed patients. Two recent large genome-wide association study (GWAS) conducted in Turkey and Japan reported association between single nucleotide polymorphism (SNP) of interleukin (IL)-10 and IL-23R/IL-12RB2 genes and BD. New insights into the perturbations of T cell homeostasis of BD recently emerged. We have recently demonstrated the promotion of Th17 responses and the suppression of regulatory T cells (Tregs) that were driven by interleukin (IL)-21 production and that correlates with BD activity. Inflammatory cells within BD inflammatory lesions included mostly neutrophils, Th1 and Th17 cells, and cytotoxic CD8+ and Î³Î´ T cells. Altogether, the recent progresses in the knowledge of BD pathogenesis pave the way for innovative therapy.
File Format	HTM / HTML
ISSN	15689972
Issue Number	10
Volume Number	11
e-ISSN	18730183
Journal	Autoimmunity Reviews
Language	English
Publisher	Elsevier
Publisher Date	2012-08-01
Publisher Place	Netherlands
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Immunology Behcet Syndrome Genetics Immunology Genetic Predisposition To Disease Autoantibodies Biosynthesis Pathology Hla-b51 Antigen Physiology Humans Inflammation Receptors, Antigen, T-cell, Gamma-delta Recurrence T-lymphocyte Subsets Metabolism Journal Article Review
Content Type	Text
Resource Type	Article
Subject	Immunology and Allergy Immunology

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