Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Neurology: Neuroimmunology & Neuroinflammation COVID-19 Article Hub
    • Diversity, Equity, & Inclusion (DEI)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit New Manuscript
    • Submit Revised Manuscript
    • Author Center

Advanced Search

Main menu

  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Neurology: Neuroimmunology & Neuroinflammation COVID-19 Article Hub
    • Diversity, Equity, & Inclusion (DEI)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit New Manuscript
    • Submit Revised Manuscript
    • Author Center
  • Home
  • Articles
  • Issues
  • COVID-19 Article Hub
  • Infographics & Video Summaries

User menu

  • My Alerts
  • Log in

Search

  • Advanced search
Neurology Neuroimmunology & Neuroinflammation
Home
A peer-reviewed clinical and translational neurology open access journal
  • My Alerts
  • Log in
Site Logo
  • Home
  • Articles
  • Issues
  • COVID-19 Article Hub
  • Infographics & Video Summaries

Share

May 2019; 6 (3) ArticleOpen Access

PD1 pathway in immune-mediated myopathies

Pathogenesis of dysfunctional T cells revisited

Samuel Knauss, Corinna Preusse, Yves Allenbach, Sarah Leonard-Louis, Mehdi Touat, Norina Fischer, Helena Radbruch, Ronja Mothes, Vitali Matyash, Wolfgang Böhmerle, Matthias Endres, Hans-Hilmar Goebel, Olivier Benveniste, Werner Stenzel
First published April 10, 2019, DOI: https://doi.org/10.1212/NXI.0000000000000558
Samuel Knauss
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Corinna Preusse
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Yves Allenbach
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sarah Leonard-Louis
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Mehdi Touat
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Norina Fischer
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Helena Radbruch
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ronja Mothes
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Vitali Matyash
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Wolfgang Böhmerle
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Matthias Endres
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Hans-Hilmar Goebel
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Olivier Benveniste
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Werner Stenzel
From the Department of Neurology (S.K., W.B., M.E.) and Department of Neuropathology (C.P., N.F., H.R., R.M., V.M., H.-H.G., W.S.), Charité—Universitätsmedizin, Berlin, Germany; Department of Internal Medicine and Clinical Immunology (Y.A., O.B.), Assistance Public-Hôpitaux de Paris, Sorbonne-Université, INSERM, UMR974, Pitié-Salpêtrière University Hospital; Unité de Pathologie Neuromusculaire (S.L.-L.), Centre de Référence Paris-Est, Groupe Hospitalier Pitié-Salpêtrière; Service de Neurologie 2-Mazarin (M.T.), Hôpitaux Universitaires La Pitié Salpêtrière—Charles Foix, APHP; OncoNeuroTox Group (M.T.), Center for Patients with Neurological Complications of Oncologic Treatments, Hôpitaux Universitaires Pitié-Salpetrière-Charles Foix et Hôpital Percy; Inserm U 1127 (M.T.), CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Université, Paris, France; Leibniz ScienceCampus Chronic Inflammation (H.R., R.M., W.S.); Center for Stroke Research Berlin (M.E.), Charité—Universitätsmedizin, Berlin; German Centre for Cardiovascular Research (DZHK) (M.E.); and German Center for Neurodegenerative Diseases (DZNE) (M.E.).
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Full PDF
Citation
PD1 pathway in immune-mediated myopathies
Pathogenesis of dysfunctional T cells revisited
Samuel Knauss, Corinna Preusse, Yves Allenbach, Sarah Leonard-Louis, Mehdi Touat, Norina Fischer, Helena Radbruch, Ronja Mothes, Vitali Matyash, Wolfgang Böhmerle, Matthias Endres, Hans-Hilmar Goebel, Olivier Benveniste, Werner Stenzel
Neurol Neuroimmunol Neuroinflamm May 2019, 6 (3) e558; DOI: 10.1212/NXI.0000000000000558

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Permissions

Make Comment

See Comments

Downloads
1187

Share

  • Article
  • Figures & Data
  • Info & Disclosures
Loading

Abstract

Objective To investigate the relevance of dysfunctional T cells in immune-mediated myopathies. We analyzed T-cell exhaustion and senescence, in the context of programmed cell death protein 1 (PD1)-related immunity in skeletal muscle biopsies from patients with immune-mediated necrotizing myopathy (IMNM), sporadic inclusion body myositis (sIBM), and myositis induced by immune checkpoint inhibitors (irMyositis).

Methods Skeletal muscle biopsies from 12 patients with IMNM, 7 patients with sIBM, and 8 patients with irMyositis were analyzed by immunostaining and immunofluorescence as well as by quantitative PCR. Eight biopsies from nondisease participants served as controls.

Results CD3+CD8+ T cells in biopsies from IMNM, sIBM, and irMyositis were largely PD1-positive, while CD68+ macrophages were sparsely positive to the ligand of programmed cell death protein 1 (PD-L1). The sarcolemma of myofibers was PD-L2+ and was colocalized with major histocompatibility complex (MHC) class I. CD68+ macrophages were colocalized with PD-L2. Senescent T cells were strongly enriched in skeletal muscle of sIBM, revealing a distinct immunologic signature. Biopsies from patients with irMyositis showed mild signs of senescence and exhaustion.

Conclusion Persistent exposure to antigens in IMNMs and sIBM may lead to T-cell exhaustion, a process controlled by the PD1 receptor and its cognate ligands PD-L1/PD-L2. To our knowledge, these data are the first evidence of presence of dysfunctional T cells and relevance of the PD1 pathway in IMNM, sIBM, and irMyositis. These findings may guide the way to a novel understanding of the immune pathogenesis of immune-mediated myopathies.

Glossary

CTL=
cytotoxic T cell;
HMGCR=
3-hydroxy-3-methylglutaryl-coenzyme A reductase;
ICI=
immune checkpoint inhibitors;
IMNM=
immune mediated necrotizing myopathy;
IFNγ=
gamma interferon;
irMyositis=
myositis induced by immune checkpoint inhibitors;
MHC=
major histocompatibility complex;
mRNA=
messanger ribonuclein acid;
MSA=
myositis-specific antibody;
PD1=
programmed cell death protein 1;
PD-L1/2=
ligand of programmed cell death protein 1/2;
qPCR=
quantitative PCR;
sIBM=
sporadic Inclusion body myositis;
SRP=
signal recognition particle

Footnotes

  • Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/NN.

  • The Article Processing Charge was funded by the authors.

  • Received September 7, 2018.
  • Accepted in final form January 23, 2019.
  • Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

View Full Text

Letters: Rapid online correspondence

No comments have been published for this article.
Comment

REQUIREMENTS

If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org

Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.

If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.

Submission specifications:

  • Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
  • Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
  • Submit only on articles published within 6 months of issue date.
  • Do not be redundant. Read any comments already posted on the article prior to submission.
  • Submitted comments are subject to editing and editor review prior to posting.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Publishing Agreement Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
  • Article
    • Abstract
    • Glossary
    • Methods
    • Results
    • Discussion
    • Authors contributions
    • Study funding
    • Disclosure
    • Footnotes
    • References
  • Figures & Data
  • Info & Disclosures
Advertisement

Use of Whole-Genome Sequencing for Mitochondrial Disease Diagnosis

Dr. Robert Pitceathly and Dr. William Macken

► Watch

Related Articles

  • No related articles found.

Topics Discussed

  • All Immunology
  • All Neuromuscular Disease
  • Chemotherapy-tumor

Alert Me

  • Alert me when eletters are published

Recommended articles

  • What's Happening
    What's happening in Neurology® Neuroimmunology & Neuroinflammation
    et al.
    Neurology, October 28, 2019
  • Article
    Inflammatory myopathy with myasthenia gravis
    Thymoma association and polymyositis pathology
    Naohiro Uchio, Kenichiro Taira, Chiseko Ikenaga et al.
    Neurology: Neuroimmunology & Neuroinflammation, December 24, 2018
  • Article
    Immune checkpoint inhibitor-related myositis and myocarditis in patients with cancer
    Mehdi Touat, Thierry Maisonobe, Samuel Knauss et al.
    Neurology, August 08, 2018
  • Article
    Necrosis in anti-SRP+ and anti-HMGCR+myopathies
    Role of autoantibodies and complement
    Yves Allenbach, Louiza Arouche-Delaperche, Corinna Preusse et al.
    Neurology, January 12, 2018
Neurology - Neuroimmunology Neuroinflammation: 10 (3)

Articles

  • Articles
  • Issues
  • Popular Articles

About

  • About the Journals
  • Ethics Policies
  • Editors & Editorial Board
  • Contact Us
  • Advertise

Submit

  • Author Center
  • Submit a Manuscript
  • Information for Reviewers
  • AAN Guidelines
  • Permissions

Subscribers

  • Subscribe
  • Sign up for eAlerts
  • RSS Feed
Site Logo
  • Visit neurology Template on Facebook
  • Follow neurology Template on Twitter
  • Visit Neurology on YouTube
  • Neurology
  • Neurology: Clinical Practice
  • Neurology: Education
  • Neurology: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology: Neuroimmunology & Neuroinflammation
Online ISSN: 2332-7812

© 2023 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise