{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T19:11:46Z","timestamp":1790190706571,"version":"4.1.0"},"reference-count":132,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2022,4,4]],"date-time":"2022-04-04T00:00:00Z","timestamp":1649030400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Myasthenia gravis (MG) is a rare autoimmune disease mediated by antibodies against components of the neuromuscular junction, particularly the acetylcholine receptor (AChR). The thymus plays a primary role in AChR-MG patients. In early-onset AChR-MG and thymoma-associated MG, an interferon type I (IFN-I) signature is clearly detected in the thymus. The origin of this chronic IFN-I expression in the thymus is not yet defined. IFN-I subtypes are normally produced in response to viral infection. However, genetic diseases called interferonopathies are associated with an aberrant chronic production of IFN-I defined as sterile inflammation. Some systemic autoimmune diseases also share common features with interferonopathies. This review aims to analyze the pathogenic role of IFN-I in these diseases as compared to AChR-MG in order to determine if AChR-MG could be an acquired interferonopathy.<\/jats:p>","DOI":"10.3390\/cells11071218","type":"journal-article","created":{"date-parts":[[2022,4,4]],"date-time":"2022-04-04T09:49:41Z","timestamp":1649065781000},"page":"1218","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Myasthenia Gravis: An Acquired Interferonopathy?"],"prefix":"10.3390","volume":"11","author":[{"given":"Clo\u00e9 A.","family":"Payet","sequence":"first","affiliation":[{"name":"Sorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Axel","family":"You","sequence":"additional","affiliation":[{"name":"Sorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Odessa-Maud","family":"Fayet","sequence":"additional","affiliation":[{"name":"Sorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadine","family":"Dragin","sequence":"additional","affiliation":[{"name":"Sorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonia","family":"Berrih-Aknin","sequence":"additional","affiliation":[{"name":"Sorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7522-5974","authenticated-orcid":false,"given":"Rozen","family":"Le Panse","sequence":"additional","affiliation":[{"name":"Sorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.jaut.2013.12.001","article-title":"Genetic Basis of Myasthenia Gravis\u2014A Comprehensive Review","volume":"52","author":"Avidan","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41572-019-0079-y","article-title":"Myasthenia Gravis","volume":"5","author":"Gilhus","year":"2019","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_3","first-page":"e1171","article-title":"Clinical and Therapeutic Features of Myasthenia Gravis in Adults Based on Age at Onset","volume":"94","author":"Segovia","year":"2020","journal-title":"Neurology"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1016\/S0140-6736(75)92779-8","article-title":"Humoral Antibodies to Acetylcholine Receptor in Patients with Myastenia Gravis","volume":"306","author":"Aharonov","year":"1975","journal-title":"Lancet"},{"key":"ref_5","first-page":"123","article-title":"Late-Onset Non-Thymomatous Myasthenia Gravis: Comparison with Early-Onset and Very Late-Onset Myasthenia Gravis","volume":"22","author":"Cho","year":"2017","journal-title":"Neurol. Asia"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1016\/0022-510X(83)90095-3","article-title":"Anti-Acetylcholine Receptor Antibodies in Myasthenia Gravis: Part 1. Relation to Clinical Parameters in 250 Patients","volume":"58","author":"Limburg","year":"1983","journal-title":"J. Neurol. Sci."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1038\/85520","article-title":"Auto-Antibodies to the Receptor Tyrosine Kinase MuSK in Patients with Myasthenia Gravis without Acetylcholine Receptor Antibodies","volume":"7","author":"Hoch","year":"2001","journal-title":"Nat. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1002\/ana.22312","article-title":"Autoantibodies to Low-Density Lipoprotein Receptor\u2013Related Protein 4 in Myasthenia Gravis","volume":"69","author":"Higuchi","year":"2011","journal-title":"Ann. Neurol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1016\/j.jneuroim.2008.01.013","article-title":"Clinical Fluctuations in MuSK Myasthenia Gravis Are Related to Antigen-Specific IgG4 Instead of IgG1","volume":"195","author":"Niks","year":"2008","journal-title":"J. Neuroimmunol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/j.jaut.2013.12.004","article-title":"A Comprehensive Analysis of the Epidemiology and Clinical Characteristics of Anti-LRP4 in Myasthenia Gravis","volume":"52","author":"Zisimopoulou","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"212","DOI":"10.3389\/fimmu.2020.00212","article-title":"Autoantibody Specificities in Myasthenia Gravis; Implications for Improved Diagnostics and Therapeutics","volume":"11","author":"Lazaridis","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"620894","DOI":"10.3389\/fimmu.2020.620894","article-title":"Non-Epithelial Thymic Stromal Cells: Unsung Heroes in Thymus Organogenesis and T Cell Development","volume":"11","author":"Nitta","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/nri3667","article-title":"Positive and Negative Selection of the T Cell Repertoire: What Thymocytes See (and Don\u2019t See)","volume":"14","author":"Klein","year":"2014","journal-title":"Nat. Rev. Immunol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"3752","DOI":"10.4049\/jimmunol.157.8.3752","article-title":"Expression of Acetylcholine Receptor Genes in Human Thymic Epithelial Cells: Implications for Myasthenia Gravis","volume":"157","author":"Wakkach","year":"1996","journal-title":"J. Immunol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/j.jneuroim.2003.11.013","article-title":"Thymic Myoid Cells Express High Levels of Muscle Genes","volume":"148","author":"Bismuth","year":"2004","journal-title":"J. Neuroimmunol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1002\/ana.20386","article-title":"Fewer Thymic Changes in MuSK Antibody-Positive than in MuSK Antibody-Negative MG","volume":"57","author":"Leite","year":"2005","journal-title":"Ann. Neurol."},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Koneczny, I., Cossins, J., Waters, P., Beeson, D., and Vincent, A. (2013). MuSK Myasthenia Gravis IgG4 Disrupts the Interaction of LRP4 with MuSK but Both IgG4 and IgG1-3 Can Disperse Preformed Agrin-Independent AChR Clusters. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0080695"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1016\/j.it.2009.04.003","article-title":"Thymic Involution and Immune Reconstitution","volume":"30","author":"Lynch","year":"2009","journal-title":"Trends Immunol."},{"key":"ref_19","first-page":"462","article-title":"Microenvironments in the Normal Thymus and the Thymus in Myasthenia Gravis","volume":"119","author":"Bofill","year":"1985","journal-title":"Am. J. Pathol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1111\/j.1749-6632.1987.tb51282.x","article-title":"The Role of the Thymus in Myasthenia Gravis: Immunohistological and Immunological Studies in 115 Casesa","volume":"505","author":"Morel","year":"1987","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1007\/s12016-016-8558-3","article-title":"Thymic Germinal Centers and Corticosteroids in Myasthenia Gravis: An Immunopathological Study in 1035 Cases and a Critical Review","volume":"52","author":"Truffault","year":"2017","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"893","DOI":"10.2353\/ajpath.2007.070240","article-title":"Myasthenia Gravis Thymus","volume":"171","author":"Leite","year":"2007","journal-title":"Am. J. Pathol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1196\/annals.1405.021","article-title":"Autoimmunizing Mechanisms in Thymoma and Thymus","volume":"1132","author":"Willcox","year":"2008","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1016\/j.imbio.2012.05.006","article-title":"SDF-1\/CXCL12 Recruits B Cells and Antigen-Presenting Cells to the Thymus of Autoimmune Myasthenia Gravis Patients","volume":"218","author":"Weiss","year":"2013","journal-title":"Immunobiology"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1002\/ana.21628","article-title":"CCL21 Overexpressed on Lymphatic Vessels Drives Thymic Hyperplasia in Myasthenia","volume":"66","author":"Ruhlmann","year":"2009","journal-title":"Ann. Neurol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.jaut.2013.12.013","article-title":"Innate Immunity in Myasthenia Gravis Thymus: Pathogenic Effects of Toll-like Receptor 4 Signaling on Autoimmunity","volume":"52","author":"Cordiglieri","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1182\/blood-2005-06-2383","article-title":"The Chemokine CXCL13 Is a Key Molecule in Autoimmune Myasthenia Gravis","volume":"108","author":"Meraouna","year":"2006","journal-title":"Blood"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1016\/j.jneuroim.2005.09.001","article-title":"Altered Expression of Chemokine Receptor CXCR5 on T Cells of Myasthenia Gravis Patients","volume":"170","author":"Saito","year":"2005","journal-title":"J. Neuroimmunol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"809","DOI":"10.3389\/fimmu.2020.00809","article-title":"CD4+ T Cells of Myasthenia Gravis Patients Are Characterized by Increased IL-21, IL-4, and IL-17A Productions and Higher Presence of PD-1 and ICOS","volume":"11","author":"Durmus","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"5324","DOI":"10.4049\/jimmunol.174.9.5324","article-title":"Overexpression of IFN-Induced Protein 10 and Its Receptor CXCR3 in Myasthenia Gravis","volume":"174","author":"Feferman","year":"2005","journal-title":"J. Immunol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1309\/CAV8RNU5TKNACKNC","article-title":"Thymoma Classification: Current Status and Future Trends","volume":"125","author":"Suster","year":"2006","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1093\/ejcts\/ezs663","article-title":"Thymoma and the Increased Risk of Developing Extrathymic Malignancies: A Multicentre Study","volume":"44","author":"Filosso","year":"2013","journal-title":"Eur. J. Cardiothorac. Surg."},{"key":"ref_33","first-page":"474512","article-title":"Thymoma in Myasthenia Gravis: From Diagnosis to Treatment","volume":"2011","author":"Romi","year":"2011","journal-title":"Autoimmune Dis."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"S266","DOI":"10.1097\/JTO.0b013e3181f1f63f","article-title":"The Autoimmune Regulator AIRE in Thymoma Biology: Autoimmunity and Beyond","volume":"5","author":"Marx","year":"2010","journal-title":"J. Thorac. Oncol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1046\/j.1365-2249.2003.02113.x","article-title":"Anti-Cytokine Autoantibodies in Autoimmunity: Preponderance of Neutralizing Autoantibodies against Interferon-Alpha, Interferon-Omega and Interleukin-12 in Patients with Thymoma and\/or Myasthenia Gravis","volume":"132","author":"Meager","year":"2003","journal-title":"Clin. Exp. Immunol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1093\/intimm\/dxg088","article-title":"Spontaneous Production of Anti-IFN-\u03b1 and Anti-IL-12 Autoantibodies by Thymoma Cells from Myasthenia Gravis Patients Suggests Autoimmunization in the Tumor","volume":"15","author":"Shiono","year":"2003","journal-title":"Int. Immunol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1002\/ana.20340","article-title":"Selective Loss of Regulatory T Cells in Thymomas","volume":"56","author":"Rosenwald","year":"2004","journal-title":"Ann. Neurol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"102337","DOI":"10.1016\/j.jaut.2019.102337","article-title":"Risk Factors Associated with Myasthenia Gravis in Thymoma Patients: The Potential Role of Thymic Germinal Centers","volume":"106","author":"Lefeuvre","year":"2020","journal-title":"J. Autoimmun."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/S0888-7543(05)80290-3","article-title":"Genetic and Physical Map of the Interferon Region on Chromosome 9p","volume":"14","author":"Fountain","year":"1992","journal-title":"Genomics"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1038\/nrd2422","article-title":"Interferons at Age 50: Past, Current and Future Impact on Biomedicine","volume":"6","author":"Borden","year":"2007","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1016\/S0079-6603(08)61008-9","article-title":"New and Atypical Families of Type I Interferons in Mammals: Comparative Functions, Structures, and Evolutionary Relationships1","volume":"Volume 56","author":"Liu","year":"1997","journal-title":"Progress in Nucleic Acid Research and Molecular Biology"},{"key":"ref_42","unstructured":"de Maeyer, E., and de Maeyer-Guignard, J. (2000). Type I Interferons. Cytokines and Cytokine Receptors, CRC Press."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1038\/11360","article-title":"Plasmacytoid Monocytes Migrate to Inflamed Lymph Nodes and Produce Large Amounts of Type I Interferon","volume":"5","author":"Cella","year":"1999","journal-title":"Nat. Med."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1016\/j.coviro.2011.10.026","article-title":"Type I Interferons: Diversity of Sources, Production Pathways and Effects on Immune Responses","volume":"1","author":"Swiecki","year":"2011","journal-title":"Curr. Opin. Virol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"778","DOI":"10.3389\/fimmu.2019.00778","article-title":"Sources of Type I Interferons in Infectious Immunity: Plasmacytoid Dendritic Cells Not Always in the Driver\u2019s Seat","volume":"10","author":"Ali","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1016\/j.cell.2006.02.015","article-title":"Pathogen Recognition and Innate Immunity","volume":"124","author":"Akira","year":"2006","journal-title":"Cell"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1038\/s41577-019-0215-7","article-title":"DAMP-Sensing Receptors in Sterile Inflammation and Inflammatory Diseases","volume":"20","author":"Gong","year":"2020","journal-title":"Nat. Rev. Immunol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"826","DOI":"10.1038\/nri2873","article-title":"Sterile Inflammation: Sensing and Reacting to Damage","volume":"10","author":"Chen","year":"2010","journal-title":"Nat. Rev. Immunol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1084\/jem.20161451","article-title":"Detection of Interferon Alpha Protein Reveals Differential Levels and Cellular Sources in Disease","volume":"214","author":"Rodero","year":"2017","journal-title":"J. Exp. Med."},{"key":"ref_50","doi-asserted-by":"crossref","unstructured":"Pitha, P.M. (2007). The Receptor of the Type I Interferon Family. Interferon: The 50th Anniversary, Springer.","DOI":"10.1007\/978-3-540-71329-6"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1111\/imr.12001","article-title":"Structural and Dynamic Determinants of Type I Interferon Receptor Assembly and Their Functional Interpretation","volume":"250","author":"Piehler","year":"2012","journal-title":"Immunol. Rev."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"615603","DOI":"10.3389\/fimmu.2020.615603","article-title":"Interferon Receptor Trafficking and Signaling: Journey to the Cross Roads","volume":"11","author":"Zanin","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"e2444","DOI":"10.1038\/cddis.2016.326","article-title":"Multiple Functions of USP18","volume":"7","author":"Honke","year":"2016","journal-title":"Cell Death Dis."},{"key":"ref_54","doi-asserted-by":"crossref","unstructured":"Fran\u00e7ois-Newton, V., de Freitas Almeida, G.M., Payelle-Brogard, B., Monneron, D., Pichard-Garcia, L., Piehler, J., Pellegrini, S., and Uz\u00e9, G. (2011). USP18-Based Negative Feedback Control Is Induced by Type I and Type III Interferons and Specifically Inactivates Interferon \u03b1 Response. PLoS ONE, 6.","DOI":"10.1371\/journal.pone.0022200"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"334","DOI":"10.3389\/fimmu.2015.00334","article-title":"MicroRNA as Type I Interferon-Regulated Transcripts and Modulators of the Innate Immune Response","volume":"6","author":"Forster","year":"2015","journal-title":"Front. Immunol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1038\/ni.2193","article-title":"The Thymic Epithelial MicroRNA Network Elevates the Threshold for Infection-Associated Thymic Involution via MiR-29a Mediated Suppression of the IFN-\u03b1 Receptor","volume":"13","author":"Papadopoulou","year":"2012","journal-title":"Nat. Immunol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"3356","DOI":"10.1128\/JVI.03085-14","article-title":"Hepatitis C Virus-Mediated Enhancement of MicroRNA MiR-373 Impairs the JAK\/STAT Signaling Pathway","volume":"89","author":"Mukherjee","year":"2015","journal-title":"J. Virol."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1002\/art.24436","article-title":"MicroRNA-146A Contributes to Abnormal Activation of the Type I Interferon Pathway in Human Lupus by Targeting the Key Signaling Proteins","volume":"60","author":"Tang","year":"2009","journal-title":"Arthritis Rheum."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"6226","DOI":"10.4049\/jimmunol.1000491","article-title":"Inducible MicroRNA-155 Feedback Promotes Type I IFN Signaling in Antiviral Innate Immunity by Targeting Suppressor of Cytokine Signaling 1","volume":"185","author":"Wang","year":"2010","journal-title":"J. Immunol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1146\/annurev-virology-092818-015756","article-title":"Interferon-Stimulated Genes: What Do They All Do","volume":"6","author":"Schoggins","year":"2019","journal-title":"Annu. Rev. Virol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"15623","DOI":"10.1073\/pnas.95.26.15623","article-title":"Identification of Genes Differentially Regulated by Interferon \u03b1, \u03b2, or \u03b3 Using Oligonucleotide Arrays","volume":"95","author":"Der","year":"1998","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1189\/jlb.69.6.912","article-title":"Functional Classification of Interferon-Stimulated Genes Identified Using Microarrays","volume":"69","author":"Holko","year":"2001","journal-title":"J. Leukoc. Biol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1314","DOI":"10.1084\/jem.147.5.1314","article-title":"Anti-Viral Activity Induced by Culturing Lymphocytes with Tumor-Derived or Virus-Transformed Cells. Enhancement of Human Natural Killer Cell Activity by Interferon and Antagonistic Inhibition of Susceptibility of Target Cells to Lysis","volume":"147","author":"Trinchieri","year":"1978","journal-title":"J. Exp. Med."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1016\/S1043-4666(03)00226-6","article-title":"IFN\u03b1 Regulates NK Cell Cytotoxicity through STAT1 Pathway","volume":"23","author":"Liang","year":"2003","journal-title":"Cytokine"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1111\/j.1365-2249.2012.04638.x","article-title":"Type I Interferon Induces CX3CL1 (Fractalkine) and CCL5 (RANTES) Production in Human Pulmonary Vascular Endothelial Cells","volume":"170","author":"Nakano","year":"2012","journal-title":"Clin. Exp. Immunol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"1216","DOI":"10.1001\/archneurol.2009.138","article-title":"Enhancement of Chemokine Expression by Interferon Beta Therapy in Patients with Multiple Sclerosis","volume":"66","author":"Cepok","year":"2009","journal-title":"Arch. Neurol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1073\/pnas.73.4.1284","article-title":"Interferon Treatment of Mice: Enhanced Expression of Histocompatibility Antigens on Lymphoid Cells","volume":"73","author":"Lindahl","year":"1976","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1084\/jem.20050821","article-title":"Type I Interferons Act Directly on CD8 T Cells to Allow Clonal Expansion and Memory Formation in Response to Viral Infection","volume":"202","author":"Kolumam","year":"2005","journal-title":"J. Exp. Med."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1084\/jem.189.3.521","article-title":"Type I Interferons Keep Activated T Cells Alive","volume":"189","author":"Marrack","year":"1999","journal-title":"J. Exp. Med."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1084\/jem.187.1.79","article-title":"Impairment of T and B Cell Development by Treatment with a Type I Interferon","volume":"187","author":"Lin","year":"1998","journal-title":"J. Exp. Med."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1016\/S1074-7613(01)00126-1","article-title":"Type I Interferons Potently Enhance Humoral Immunity and Can Promote Isotype Switching by Stimulating Dendritic Cells In Vivo","volume":"14","author":"Schiavoni","year":"2001","journal-title":"Immunity"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1111\/j.1749-6632.2011.06220.x","article-title":"Type I Interferonopathies: A Novel Set of Inborn Errors of Immunity","volume":"1238","author":"Crow","year":"2011","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1002\/ana.410150109","article-title":"A Progressive Familial Encephalopathy in Infancy with Calcifications of the Basal Ganglia and Chronic Cerebrospinal Fluid Lymphocytosis","volume":"15","author":"Aicardi","year":"1984","journal-title":"Ann. Neurol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1007\/s12519-019-00273-z","article-title":"Toward a Better Understanding of Type I Interferonopathies: A Brief Summary, Update and Beyond","volume":"16","author":"Yu","year":"2020","journal-title":"World J. Pediatr."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/0022-510X(88)90125-6","article-title":"Intrathecal Synthesis of Interferon-Alpha in Infants with Progressive Familial Encephalopathy","volume":"84","author":"Lebon","year":"1988","journal-title":"J. Neurol. Sci."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1159","DOI":"10.1016\/S1474-4422(13)70258-8","article-title":"Assessment of Interferon-Related Biomarkers in Aicardi-Gouti\u00e8res Syndrome Associated with Mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, and ADAR: A Case-Control Study","volume":"12","author":"Rice","year":"2013","journal-title":"Lancet Neurol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"A47","DOI":"10.1053\/ejpn.2002.0574","article-title":"Interferon and Aicardi-Gouti\u00e8res Syndrome","volume":"6","author":"Lebon","year":"2002","journal-title":"Eur. J. Paediatr. Neurol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1084\/jem.20021553","article-title":"Interferon and Granulopoiesis Signatures in Systemic Lupus Erythematosus Blood","volume":"197","author":"Bennett","year":"2003","journal-title":"J. Exp. Med."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.jaut.2013.01.008","article-title":"Genetic Susceptibility to SLE: Recent Progress from GWAS","volume":"41","author":"Cui","year":"2013","journal-title":"J. Autoimmun."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1016\/j.autrev.2012.10.006","article-title":"Type I Interferons in Sj\u00f6gren\u2019s Syndrome","volume":"12","author":"Yao","year":"2013","journal-title":"Autoimmun. Rev."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1136\/annrheumdis-2012-202753","article-title":"The Type I IFN Signature as a Biomarker of Preclinical Rheumatoid Arthritis","volume":"72","author":"Brink","year":"2013","journal-title":"Ann. Rheum. Dis."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1486","DOI":"10.1136\/ard.2007.086421","article-title":"Type I Interferons Might Form the Link between Toll-like Receptor (TLR) 3\/7 and TLR4-Mediated Synovial Inflammation in Rheumatoid Arthritis (RA)","volume":"68","author":"Roelofs","year":"2009","journal-title":"Ann. Rheum. Dis."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1002\/ana.20464","article-title":"Interferon-\u03b1\/\u03b2\u2013Mediated Innate Immune Mechanisms in Dermatomyositis","volume":"57","author":"Greenberg","year":"2005","journal-title":"Ann. Neurol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1111\/j.1472-8206.2011.00929.x","article-title":"Systemic Lupus Erythematosus Induced by Interferon \u03921 Therapy in a Patient with Multiple Sclerosis","volume":"26","author":"Bahri","year":"2012","journal-title":"Fundam. Clin. Pharmacol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"1825","DOI":"10.1016\/S0140-6736(05)79887-1","article-title":"Subacute Cutaneous Lupus Erythematosus Associated with Interferon Beta-1a","volume":"352","author":"Nousari","year":"1998","journal-title":"Lancet"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"e3782","DOI":"10.1097\/MD.0000000000003782","article-title":"Myasthenia Crisis Induced by Pegylated-Interferon in Patient with Chronic Hepatitis C","volume":"95","author":"Baik","year":"2016","journal-title":"Medicine"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1136\/jnnp.2003.028233","article-title":"Development of Myasthenia Gravis in Two Patients with Multiple Sclerosis Following Interferon \u03b2 Treatment","volume":"75","author":"Dionisiotis","year":"2004","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_88","first-page":"75","article-title":"Development of Myasthenia Gravis after Interferon Alpha Therapy","volume":"39","author":"Gurtubay","year":"1999","journal-title":"Electromyogr. Clin. Neurophysiol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"25","DOI":"10.2147\/JIR.S9480","article-title":"Injectable Interferon Beta-1b for the Treatment of Relapsing Forms of Multiple Sclerosis","volume":"3","author":"Jankovic","year":"2010","journal-title":"J. Inflamm. Res."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"1227","DOI":"10.1016\/j.imbio.2016.06.012","article-title":"A Novel Infection- and Inflammation-Associated Molecular Signature in Peripheral Blood of Myasthenia Gravis Patients","volume":"221","author":"Barzago","year":"2016","journal-title":"Immunobiology"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.3389\/fneur.2019.01343","article-title":"The Muscle Is Not a Passive Target in Myasthenia Gravis","volume":"10","author":"Vilquin","year":"2019","journal-title":"Front. Neurol."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"5941","DOI":"10.4049\/jimmunol.174.10.5941","article-title":"Effects of Cytokines on Acetylcholine Receptor Expression: Implications for Myasthenia Gravis","volume":"174","author":"Christadoss","year":"2005","journal-title":"J. Immunol."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1002\/ana.23791","article-title":"Implication of Double-Stranded RNA Signaling in the Etiology of Autoimmune Myasthenia Gravis","volume":"73","author":"Cufi","year":"2013","journal-title":"Ann. Neurol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.jaut.2013.12.015","article-title":"Both Treg Cells and Tconv Cells Are Defective in the Myasthenia Gravis Thymus: Roles of IL-17 and TNF-\u03b1","volume":"52","author":"Gradolatto","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/j.jaut.2013.12.016","article-title":"Central Role of Interferon-Beta in Thymic Events Leading to Myasthenia Gravis","volume":"52","author":"Cufi","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1007\/BF00918185","article-title":"In Vitro Interleukin-1 (IL-1) Production in Thymic Hyperplasia and Thymoma from Patients with Myasthenia Gravis","volume":"11","author":"Aime","year":"1991","journal-title":"J. Clin. Immunol."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.jaut.2018.11.005","article-title":"Il-23\/Th17 Cell Pathway: A Promising Target to Alleviate Thymic Inflammation Maintenance in Myasthenia Gravis","volume":"98","author":"Villegas","year":"2019","journal-title":"J. Autoimmun."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.jaut.2013.12.018","article-title":"Thymoma-Associated Myasthenia Gravis: On the Search for a Pathogen Signature","volume":"52","author":"Cufi","year":"2014","journal-title":"J. Autoimmun."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"4564","DOI":"10.1128\/MCB.00146-10","article-title":"The Type I Interferon Signaling Pathway Is a Target for Glucocorticoid Inhibition","volume":"30","author":"Flammer","year":"2010","journal-title":"Mol. Cell. Biol."},{"key":"ref_100","first-page":"57","article-title":"Thymus and Myasthenia Gravis: What Can We Learn from DNA Microarrays","volume":"201\u2013202","author":"Meraouna","year":"2008","journal-title":"J. Neuroimmunol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1056\/NEJMoa1602489","article-title":"Randomized Trial of Thymectomy in Myasthenia Gravis","volume":"375","author":"Wolfe","year":"2016","journal-title":"N. Engl. J. Med."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"30649","DOI":"10.1073\/pnas.2007206117","article-title":"Thymus-Derived B Cell Clones Persist in the Circulation after Thymectomy in Myasthenia Gravis","volume":"117","author":"Jiang","year":"2020","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_103","doi-asserted-by":"crossref","unstructured":"Xu, D., Staedman, A., and Zhang, L. (2013). CD20 Antibody Primes B Lymphocytes for Type I Interferon Production. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0067900"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"100590","DOI":"10.1016\/j.eclinm.2020.100590","article-title":"Eculizumab as an Emergency Treatment for Adult Patients with Severe COVID-19 in the Intensive Care Unit: A Proof-of-Concept Study","volume":"28","author":"Annane","year":"2020","journal-title":"eClinicalMedicine"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.jaut.2005.09.024","article-title":"Infections and Autoimmune Diseases","volume":"25","author":"Bach","year":"2005","journal-title":"J. Autoimmun."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"618021","DOI":"10.3389\/fneur.2021.618021","article-title":"A Systematic Review of the Potential Implication of Infectious Agents in Myasthenia Gravis","volume":"12","author":"Leopardi","year":"2021","journal-title":"Front. Neurol."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1080\/08916930802024772","article-title":"Regulation and Dysregulation of Epstein\u2013Barr Virus Latency: Implications for the Development of Autoimmune Diseases","volume":"41","author":"Niller","year":"2008","journal-title":"Autoimmunity"},{"key":"ref_108","doi-asserted-by":"crossref","unstructured":"Savino, W. (2006). The Thymus Is a Common Target Organ in Infectious Diseases. PLoS Pathog., 2.","DOI":"10.1371\/journal.ppat.0020062"},{"key":"ref_109","first-page":"385","article-title":"Epstein-Barr Virus Genome in Thymoma and Thymic Lymphoid Hyperplasia","volume":"131","author":"McGuire","year":"1988","journal-title":"Am. J. Pathol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1002\/ana.21902","article-title":"Epstein-Barr Virus Persistence and Reactivation in Myasthenia Gravis Thymus","volume":"67","author":"Cavalcante","year":"2010","journal-title":"Ann. Neurol."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1002\/ana.22488","article-title":"Intrathymic Epstein-Barr Virus Infection Is Not a Prominent Feature of Myasthenia Gravis","volume":"70","author":"Kakalacheva","year":"2011","journal-title":"Ann. Neurol."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1002\/ana.22522","article-title":"Lack of Evidence for Epstein-Barr Virus Infection in Myasthenia Gravis Thymus","volume":"70","author":"Meyer","year":"2011","journal-title":"Ann. Neurol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1111\/j.1468-1331.2011.03636.x","article-title":"High Anti-EBNA-1 IgG Levels Are Associated with Early-Onset Myasthenia Gravis","volume":"19","author":"Csuka","year":"2012","journal-title":"Eur. J. Neurol."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/s13027-019-0254-5","article-title":"Association between Epstein-Barr Virus and Thymic Epithelial Tumors: A Systematic Review","volume":"14","author":"Zhang","year":"2019","journal-title":"Infect. Agents Cancer"},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"95432","DOI":"10.18632\/oncotarget.20731","article-title":"Epstein-Barr Virus in Tumor-Infiltrating B Cells of Myasthenia Gravis Thymoma: An Innocent Bystander or an Autoimmunity Mediator","volume":"8","author":"Cavalcante","year":"2017","journal-title":"Oncotarget"},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1126\/science.abj8222","article-title":"Longitudinal Analysis Reveals High Prevalence of Epstein-Barr Virus Associated with Multiple Sclerosis","volume":"375","author":"Bjornevik","year":"2022","journal-title":"Science"},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1002\/ana.20820","article-title":"Infectious Mononucleosis and Risk for Multiple Sclerosis: A Meta-Analysis","volume":"59","author":"Thacker","year":"2006","journal-title":"Ann. Neurol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"1118","DOI":"10.1212\/WNL.0b013e3181d7d884","article-title":"Detection of Poliovirus-Infected Macrophages in Thymus of Patients with Myasthenia Gravis","volume":"74","author":"Cavalcante","year":"2010","journal-title":"Neurology"},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1038\/nri2527","article-title":"Antiviral Immune Responses: Triggers of or Triggered by Autoimmunity","volume":"9","author":"Getts","year":"2009","journal-title":"Nat. Rev. Immunol."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1016\/S0002-9440(10)62960-4","article-title":"Increased Toll-like Receptor 4 Expression in Thymus of Myasthenic Patients with Thymitis and Thymic Involution","volume":"167","author":"Bernasconi","year":"2005","journal-title":"Am. J. Pathol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1146\/annurev-immunol-032414-112240","article-title":"Innate Immune Pattern Recognition: A Cell Biological Perspective","volume":"33","author":"Brubaker","year":"2015","journal-title":"Annu. Rev. Immunol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.3389\/fimmu.2017.01029","article-title":"Use of Toll-Like Receptor Agonists to Induce Ectopic Lymphoid Structures in Myasthenia Gravis Mouse Models","volume":"8","author":"Robinet","year":"2017","journal-title":"Front. Immunol."},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1111\/nyas.13519","article-title":"Thymus Involvement in Early-Onset Myasthenia Gravis: Thymic Changes in Autoimmune Myasthenia Gravis","volume":"1412","author":"Cron","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_124","doi-asserted-by":"crossref","unstructured":"Sengupta, M., Wang, B.-D., Lee, N.H., Marx, A., Kusner, L.L., and Kaminski, H.J. (2018). MicroRNA and MRNA Expression Associated with Ectopic Germinal Centers in Thymus of Myasthenia Gravis. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0205464"},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"142","DOI":"10.3389\/fimmu.2020.00142","article-title":"MiR-146a in Myasthenia Gravis Thymus Bridges Innate Immunity with Autoimmunity and Is Linked to Therapeutic Effects of Corticosteroids","volume":"11","author":"Bortone","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1111\/cei.12265","article-title":"Delivery of an MiR155 Inhibitor by Anti-CD20 Single-Chain Antibody into B Cells Reduces the Acetylcholine Receptor-Specific Autoantibodies and Ameliorates Experimental Autoimmune Myasthenia Gravis","volume":"176","author":"Wang","year":"2014","journal-title":"Clin. Exp. Immunol."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"e164","DOI":"10.1038\/emm.2015.21","article-title":"MiR-155 Modulates the Inflammatory Phenotype of Intestinal Myofibroblasts by Targeting SOCS1 in Ulcerative Colitis","volume":"47","author":"Pathak","year":"2015","journal-title":"Exp. Mol. Med."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1186\/s12974-020-01958-3","article-title":"Decreased Expression of MiR-29 Family Associated with Autoimmune Myasthenia Gravis","volume":"17","author":"Cron","year":"2020","journal-title":"J. Neuroinflamm."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"2567","DOI":"10.12659\/MSM.894307","article-title":"PTPN22 R620W Polymorphism Is Associated with Myasthenia Gravis Risk: A Systematic Review and Meta-Analysis","volume":"21","author":"Xiong","year":"2015","journal-title":"Med. Sci. Monit."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.immuni.2013.06.013","article-title":"The Autoimmunity-Associated Gene PTPN22 Potentiates Toll-like Receptor-Driven, Type 1 Interferon-Dependent Immunity","volume":"39","author":"Wang","year":"2013","journal-title":"Immunity"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1002\/ana.23691","article-title":"Risk for Myasthenia Gravis Maps to a 151Pro\u2192Ala Change in TNIP1 and to Human Leukocyte Antigen-B*08","volume":"72","author":"Gregersen","year":"2012","journal-title":"Ann. Neurol."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"e3491269","DOI":"10.1155\/2018\/3491269","article-title":"TNIP1 in Autoimmune Diseases: Regulation of Toll-like Receptor Signaling","volume":"2018","author":"Shamilov","year":"2018","journal-title":"J. Immunol. Res."}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/7\/1218\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:49:40Z","timestamp":1760136580000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/7\/1218"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,4]]},"references-count":132,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2022,4]]}},"alternative-id":["cells11071218"],"URL":"https:\/\/doi.org\/10.3390\/cells11071218","relation":{},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,4]]}}}