THE THYMUS IN MYASTHENIA GRAVIS: SITE OF "INNATE AUTOIMMUNITY"?

被引：60

作者：

Cavalcante, Paola ^{[1
]}

Le Panse, Rozen ^{[2
]}

Berrih-Aknin, Sonia ^{[2
]}

Maggi, Lorenzo ^{[1
]}

Antozzi, Carlo ^{[1
]}

Baggi, Fulvio ^{[1
]}

Bernasconi, Pia ^{[1
]}

Mantegazza, Renato ^{[1
]}

机构：

[1] Fdn Ist Neurol Carlo Besta, Dept Neurol Neuromuscular Dis & Neuroimmunol 4, I-20133 Milan, Italy

[2] UPMC, INSERM U974, CNRS UMR7215, UMR,AIM,Ctr Chirurg Marie Lannelongue, Le Plessis Robinson, France

来源：

MUSCLE & NERVE | 2011年 / 44卷 / 04期

关键词：

inflammation; innate immunity; myasthenia gravis; thymus; viral infection; EPSTEIN-BARR-VIRUS; ANTIACETYLCHOLINE RECEPTOR ANTIBODY; MULTIPLE-SCLEROSIS BRAIN; NICOTINIC ACETYLCHOLINE-RECEPTOR; REGULATORY T-CELLS; IN-VITRO SYNTHESIS; ACTIVATED B-CELLS; MYOID CELLS; EPITHELIAL-CELLS; EXTENDED THYMECTOMY;

D O I：

10.1002/mus.22103

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Myasthenia gravis (MG) is an autoimmune disorder caused, in most cases, by autoantibodies against components of the neuromuscular junction, frequently the acetylcholine receptor (AChR), and less often the muscle-specific kinase receptor. The thymus plays a major role in the pathogenesis of MG with anti-AChR antibodies: it shows marked pathologic alterations (hyperplastic or tumoral) in most AChR-positive patients and contains the elements required to initiate and sustain an autoimmune reaction (AChR autoantigen, AChR-specific T cells, and autoantibody-secreting plasma cells). In this study we review early and more recent findings implicating the thymus as site of AChR autosensitization in MG and briefly discuss the therapeutic role of thymectomy. We also summarize data showing that the MG thymus is in a state of chronic inflammation, and we review emerging evidence of a viral contribution to the onset and maintenance of the thymic autoimmune response. Muscle Nerve 44: 467-484, 2011

引用

页码：467 / 484

页数：18

共 163 条

[1] THYMUS IN MYASTHENIA-GRAVIS - EVIDENCE FOR ALTERED CELL-POPULATIONS [J].