| Thymic remodeling associated with hyperplasia in myasthenia gravis. | |
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MedLine Citation:
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PMID: 20402580 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Acquired myasthenia gravis (MG), a neurological autoimmune disease, is caused by autoantibodies against components of the neuromuscular junction that lead to disabling muscle fatigability. The thymus is clearly involved in the pathogenesis of early-onset MG with anti-acetylcholine receptor antibodies, and thymic hyperplasia of lympho-proliferative origin is a hallmark of the disease. In this review, we describe the structural and cellular changes associated with thymic hyperplasia, its main characteristics being the development of ectopic germinal centers (GCs) associated with active neoangiogenic processes, such as development of high endothelial venules and lymphangiogenesis. What triggers such thymic abnormalities in MG is not yet clear. A thymic transcriptome analysis has demonstrated a strong inflammatory signature in MG that could orchestrate the development of thymic hyperplasia. In this context, thymic epithelial cells (TECs) seem to play a central role, either by contributing or responding to the inflammatory environment and up-regulating the autoimmune response. In particular, MG TECs clearly overexpress various cytokines, among which chemokines play a crucial role in the recruitment of peripheral lymphocytes to the thymus via the newly expanded vessel network, thereby leading to the development of ectopic GCs. Clearly, a better understanding of major events that lead to thymic hyperplasia will help optimize strategies toward more specific therapy for MG. |
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Authors:
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Rozen Le Panse; Jacky Bismuth; Géraldine Cizeron-Clairac; Julia Miriam Weiss; Perrine Cufi; Philippe Dartevelle; Nicole Kerlero De Rosbo; Sonia Berrih-Aknin |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
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Title: Autoimmunity Volume: 43 ISSN: 1607-842X ISO Abbreviation: Autoimmunity Publication Date: 2010 Aug |
Date Detail:
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Created Date: 2010-08-19 Completed Date: 2010-11-30 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8900070 Medline TA: Autoimmunity Country: England |
Other Details:
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Languages: eng Pagination: 401-12 Citation Subset: IM |
Affiliation:
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Hôpital Marie Lannelongue, CNRS UMR 8162, 92350 Le Plessis-Robinson, France. rozen.lepanse@u-psud.fr |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Autoantibodies / biosynthesis, immunology Chemokines / physiology Epithelial Cells / immunology Humans Hyperplasia Interferons / physiology Lymphangiogenesis Myasthenia Gravis / immunology, metabolism, pathology* Neovascularization, Pathologic Neuromuscular Junction / immunology, pathology Receptors, Nicotinic / immunology, metabolism Thymus Gland / immunology, pathology*, physiopathology |
| Chemical | |
Reg. No./Substance:
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0/Autoantibodies; 0/Chemokines; 0/Receptors, Nicotinic; 9008-11-1/Interferons |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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