The thymus in myasthenia gravis: Site of "innate autoimmunity"?

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 a...

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Published inMuscle & nerve Vol. 44; no. 4; pp. 467 - 484
Main Authors Cavalcante, Paola, Le Panse, Rozen, Berrih-aknin, Sonia, Maggi, Lorenzo, Antozzi, Carlo, Baggi, Fulvio, Bernasconi, Pia, Mantegazza, Renato
Format Journal Article
LanguageEnglish
Published Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.10.2011
Wiley
Subjects
Autoantibodies - immunology
Autoimmune Diseases - immunology
Autoimmune Diseases - metabolism
Autoimmune Diseases - pathology
Biological and medical sciences
Diseases of striated muscles. Neuromuscular diseases
Humans
Immunity, Innate - physiology
inflammation
innate immunity
Medical sciences
Muscle
myasthenia gravis
Myasthenia Gravis - immunology
Myasthenia Gravis - pathology
Myasthenia Gravis - therapy
Neurology
Pharmacology. Drug treatments
thymus
Thymus Gland - abnormalities
Thymus Gland - immunology
Thymus Gland - metabolism
viral infection
Autoimmunity
Human
Immunopathology
Nervous system diseases
Neuromuscular diseases
thymus
viral infection
Pathogenesis
Autoantibody
Autoimmune disease
Neuromuscular junction
Infection
Chronic
Cholinergic receptor
Myasthenia gravis
innate immunity
inflammation
Viral disease
Tumor
Muscle
Biological receptor
Online AccessGet full text
ISSN0148-639X
1097-4598
1097-4598
DOI10.1002/mus.22103

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Abstract 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, 2011
AbstractList 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.
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, 2011
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.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.
Author Cavalcante, Paola
Le Panse, Rozen
Mantegazza, Renato
Baggi, Fulvio
Berrih-aknin, Sonia
Bernasconi, Pia
Antozzi, Carlo
Maggi, Lorenzo
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  organization: Department of Neurology IV, Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy
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  organization: Unité mixte de recherche, CNRS UMR7215/INSERM U974/UPMC/AIM, Thérapie des maladies du muscle strié, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson, France
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  surname: Berrih-aknin
  fullname: Berrih-aknin, Sonia
  organization: Unité mixte de recherche, CNRS UMR7215/INSERM U974/UPMC/AIM, Thérapie des maladies du muscle strié, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson, France
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  surname: Antozzi
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  organization: Department of Neurology IV, Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy
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  givenname: Fulvio
  surname: Baggi
  fullname: Baggi, Fulvio
  organization: Department of Neurology IV, Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy
– sequence: 7
  givenname: Pia
  surname: Bernasconi
  fullname: Bernasconi, Pia
  organization: Department of Neurology IV, Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy
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  givenname: Renato
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  organization: Department of Neurology IV, Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy
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Issue 4
Keywords Autoimmunity
Human
Immunopathology
Nervous system diseases
Neuromuscular diseases
thymus
viral infection
Pathogenesis
Autoantibody
Autoimmune disease
Neuromuscular junction
Infection
Chronic
Cholinergic receptor
Myasthenia gravis
innate immunity
inflammation
Viral disease
Tumor
Muscle
Biological receptor
Language English
License http://onlinelibrary.wiley.com/termsAndConditions#vor
CC BY 4.0
Copyright © 2011 Wiley Periodicals, Inc.
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PublicationDate October 2011
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  text: October 2011
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PublicationTitle Muscle & nerve
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Publisher Wiley Subscription Services, Inc., A Wiley Company
Wiley
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Snippet Myasthenia gravis (MG) is an autoimmune disorder caused, in most cases, by autoantibodies against components of the neuromuscular junction, frequently the...
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SubjectTerms Autoantibodies - immunology
Autoimmune Diseases - immunology
Autoimmune Diseases - metabolism
Autoimmune Diseases - pathology
Biological and medical sciences
Diseases of striated muscles. Neuromuscular diseases
Humans
Immunity, Innate - physiology
inflammation
innate immunity
Medical sciences
Muscle
myasthenia gravis
Myasthenia Gravis - immunology
Myasthenia Gravis - pathology
Myasthenia Gravis - therapy
Neurology
Pharmacology. Drug treatments
thymus
Thymus Gland - abnormalities
Thymus Gland - immunology
Thymus Gland - metabolism
viral infection
Title The thymus in myasthenia gravis: Site of "innate autoimmunity"?
URI https://api.istex.fr/ark:/67375/WNG-3N6JM4QX-Q/fulltext.pdf
https://onlinelibrary.wiley.com/doi/abs/10.1002%2Fmus.22103
https://www.ncbi.nlm.nih.gov/pubmed/21922466
https://www.proquest.com/docview/890676329
Volume 44
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