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Autoimmune Diseases
Volume 2011, Article ID 213092, 17 pages
http://dx.doi.org/10.4061/2011/213092
Research Article

Inflammation and Epstein-Barr Virus Infection Are Common Features of Myasthenia Gravis Thymus: Possible Roles in Pathogenesis

1Department of Neurology IV, Neuromuscular Diseases and Neuroimmunology, Neurological Institute C. Besta Foundation, 20133 Milan, Italy
2Anatomia Patologica, Azienda Ospedaliera Bolognini Seriate, 24068 Seriate, Italy
3Unité Mixte de Recherche, CNRS UMR7215/INSERM U974/UPMC/AIM, Thérapie des Maladies du Muscle Strié, Centre Chirurgical Marie Lannelongue, 92350 Le Plessis Robinson, France

Received 30 May 2011; Accepted 23 June 2011

Academic Editor: Nils Erik Gilhus

Copyright © 2011 Paola Cavalcante et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The thymus plays a major role in myasthenia gravis (MG). Our recent finding of a persistent Epstein-Barr (EBV) virus infection in some MG thymuses, combined with data showing that the thymus is in a proinflammatory state in most patients, supports a viral contribution to the pathogenesis of MG. Aim of this study was to gain further evidence for intrathymic chronic inflammation and EBV infection in MG patients. Transcriptional profiling by low density array and real-time PCR showed overexpression of genes involved in inflammatory and immune response in MG thymuses. Real-time PCR for EBV genome, latent (EBER1, EBNA1, LMP1) and lytic (BZLF1) transcripts, and immunohistochemistry for LMP1 and BZLF1 proteins confirmed an active intrathymic EBV infection, further supporting the hypothesis that EBV might contribute to onset or perpetuation of the autoimmune response in MG. Altogether, our results support a role of inflammation and EBV infection as pathogenic features of MG thymus.