{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T17:57:26Z","timestamp":1793383046444,"version":"4.5.0"},"reference-count":209,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T00:00:00Z","timestamp":1649808000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Idiopathic inflammatory myopathies (IIM), collectively known as myositis, are a composite group of rare autoimmune diseases affecting mostly skeletal muscle, although other organs or tissues may also be involved. The main clinical feature of myositis is subacute, progressive, symmetrical muscle weakness in the proximal arms and legs, whereas subtypes of myositis may also present with extramuscular features, such as skin involvement, arthritis or interstitial lung disease (ILD). Established subgroups of IIM include dermatomyositis (DM), immune-mediated necrotizing myopathy (IMNM), anti-synthetase syndrome (ASyS), overlap myositis (OM) and inclusion body myositis (IBM). Although these subgroups have overlapping clinical features, the widespread variation in the clinical manifestations of IIM suggests different pathophysiological mechanisms. Various components of the immune system are known to be important immunopathogenic pathways in IIM, although the exact pathophysiological mechanisms causing the muscle damage remain unknown. Current treatment, which consists of glucocorticoids and other immunosuppressive or immunomodulating agents, often fails to achieve a sustained beneficial response and is associated with various adverse effects. New therapeutic targets have been identified that may improve outcomes in patients with IIM. A better understanding of the overlapping and diverging pathophysiological mechanisms of the major subgroups of myositis is needed to optimize treatment. The aim of this review is to report on recent advancements regarding DM and IMNM.<\/jats:p>","DOI":"10.3390\/ijms23084301","type":"journal-article","created":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T21:33:42Z","timestamp":1649885622000},"page":"4301","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":60,"title":["Pathophysiological Mechanisms and Treatment of Dermatomyositis and Immune Mediated Necrotizing Myopathies: A Focused Review"],"prefix":"10.3390","volume":"23","author":[{"given":"Renske G.","family":"Kamperman","sequence":"first","affiliation":[{"name":"Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, 1100 DD Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anneke J.","family":"van der Kooi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, 1100 DD Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianne","family":"de Visser","sequence":"additional","affiliation":[{"name":"Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, 1100 DD Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Aronica","sequence":"additional","affiliation":[{"name":"Department of (Neuro)Pathology, Amsterdam UMC, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joost","family":"Raaphorst","sequence":"additional","affiliation":[{"name":"Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, 1100 DD Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1528","DOI":"10.1001\/jamaneurol.2018.2598","article-title":"Development of a New Classification System for Idiopathic Inflammatory Myopathies Based on Clinical Manifestations and Myositis-Specific Autoantibodies","volume":"75","author":"Mariampillai","year":"2018","journal-title":"JAMA Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/j.nmd.2019.10.005","article-title":"239th ENMC International Workshop: Classification of dermatomyositis, Amsterdam, the Netherlands, 14\u201316 December 2018","volume":"30","author":"Mammen","year":"2020","journal-title":"Neuromuscul. Disord."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"109","DOI":"10.3233\/JND-180308","article-title":"Current Classification and Management of Inflammatory Myopathies","volume":"5","author":"Schmidt","year":"2018","journal-title":"J. Neuromuscul. Dis."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1097\/WCO.0000000000000855","article-title":"Where are we moving in the classification of idiopathic inflammatory myopathies?","volume":"33","author":"Tanboon","year":"2020","journal-title":"Curr. Opin. Neurol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1212\/WNL.61.3.316","article-title":"Polymyositis: An overdiagnosed entity","volume":"61","author":"Bronner","year":"2003","journal-title":"Neurology"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"3398","DOI":"10.1093\/rheumatology\/keaa801","article-title":"Polymyositis: Is there anything left? A retrospective diagnostic review from a tertiary myositis centre","volume":"60","author":"Lilleker","year":"2021","journal-title":"Rheumatology"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1016\/j.nmd.2004.02.006","article-title":"119th ENMC international workshop: Trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, Naarden, The Netherlands, 10\u201312 October 2003","volume":"14","author":"Hoogendijk","year":"2004","journal-title":"Neuromuscul. Disord."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.nmd.2017.09.016","article-title":"224th ENMC International Workshop:: Clinico-sero-pathological classification of immune-mediated necrotizing myopathies Zandvoort, The Netherlands, 14\u201316 October 2016","volume":"28","author":"Allenbach","year":"2018","journal-title":"Neuromuscul. Disord."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1464","DOI":"10.1378\/chest.10-0180","article-title":"Interstitial lung disease associated with the idiopathic inflammatory myopathies: What progress has been made in the past 35 years?","volume":"138","author":"Connors","year":"2010","journal-title":"Chest"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"992","DOI":"10.1007\/s00415-014-7313-z","article-title":"Long-term follow-up of 62 patients with myositis","volume":"261","author":"Hoogendijk","year":"2014","journal-title":"J. Neurol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1393","DOI":"10.1111\/1756-185X.13559","article-title":"Overlap myositis, a distinct entity beyond primary inflammatory myositis: A retrospective analysis of a large cohort from the REMICAM registry","volume":"22","author":"Joven","year":"2019","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1097\/BOR.0000000000000536","article-title":"The clinico-serological spectrum of overlap myositis","volume":"30","author":"Fredi","year":"2018","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"309","DOI":"10.55563\/clinexprheumatol\/8xj0b9","article-title":"Defining anti-synthetase syndrome: A systematic literature review","volume":"40","author":"Zanframundo","year":"2022","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1111\/j.1365-2990.2010.01153.x","article-title":"Review: An update on inflammatory and autoimmune myopathies","volume":"37","author":"Dalakas","year":"2011","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1212\/WNL.0000000000000642","article-title":"Evaluation and construction of diagnostic criteria for inclusion body myositis","volume":"83","author":"Lloyd","year":"2014","journal-title":"Neurology"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1007\/s00401-015-1384-5","article-title":"Amyloid deposits and inflammatory infiltrates in sporadic inclusion body myositis: The inflammatory egg comes before the degenerative chicken","volume":"129","author":"Benveniste","year":"2015","journal-title":"Acta Neuropathol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1002\/acn3.419","article-title":"Immune and myodegenerative pathomechanisms in inclusion body myositis","volume":"4","author":"Keller","year":"2017","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1136\/annrheumdis-2016-209247","article-title":"Consensus-based recommendations for the management of juvenile dermatomyositis","volume":"76","author":"Baildam","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1097\/BOR.0000000000000547","article-title":"Peculiar clinicopathological features of immune-mediated necrotizing myopathies","volume":"30","author":"Allenbach","year":"2018","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1002\/art.11484","article-title":"Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy","volume":"50","author":"Kao","year":"2004","journal-title":"Arthritis Rheum."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1136\/annrheumdis-2017-211868","article-title":"The EuroMyositis registry: An international collaborative tool to facilitate myositis research","volume":"77","author":"Lilleker","year":"2018","journal-title":"Ann. Rheum. Dis."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1038\/s41572-021-00321-x","article-title":"Idiopathic inflammatory myopathies","volume":"7","author":"Lundberg","year":"2021","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"R70","DOI":"10.1186\/ar2987","article-title":"Cancer risks of dermatomyositis and polymyositis: A nationwide cohort study in Taiwan","volume":"12","author":"Chen","year":"2010","journal-title":"Arthritis Res. Ther."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1016\/S0140-6736(00)03540-6","article-title":"Frequency of specific cancer types in dermatomyositis and polymyositis: A population-based study","volume":"357","author":"Hill","year":"2001","journal-title":"Lancet"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1016\/j.autrev.2016.07.023","article-title":"Anti-HMGCR antibodies as a biomarker for immune-mediated necrotizing myopathies: A history of statins and experience from a large international multi-center study","volume":"15","author":"Musset","year":"2016","journal-title":"Autoimmun. Rev."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1002\/art.30156","article-title":"Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase in patients with statin-associated autoimmune myopathy","volume":"63","author":"Mammen","year":"2011","journal-title":"Arthritis Rheum."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1097\/MD.0000000000000028","article-title":"Anti-HMGCR autoantibodies in European patients with autoimmune necrotizing myopathies: Inconstant exposure to statin","volume":"93","author":"Allenbach","year":"2014","journal-title":"Medicine"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1186\/s13075-017-1383-0","article-title":"RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis","volume":"19","author":"Gallardo","year":"2017","journal-title":"Arthritis Res. Ther."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1448","DOI":"10.3899\/jrheum.141443","article-title":"The Prevalence of Individual Histopathologic Features Varies according to Autoantibody Status in Muscle Biopsies from Patients with Dermatomyositis","volume":"42","author":"Corse","year":"2015","journal-title":"J. Rheumatol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1007\/s11926-010-0101-6","article-title":"Dermatomyositis and type 1 interferons","volume":"12","author":"Greenberg","year":"2010","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1111\/nan.12519","article-title":"Diagnostic potential of sarcoplasmic myxovirus resistance protein A expression in subsets of dermatomyositis","volume":"45","author":"Uruha","year":"2019","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"e739","DOI":"10.1212\/WNL.0000000000013176","article-title":"Dermatomyositis: Muscle Pathology According to Antibody Subtypes","volume":"98","author":"Tanboon","year":"2022","journal-title":"Neurology"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1212\/WNL.0000000000003568","article-title":"Sarcoplasmic MxA expression: A valuable marker of dermatomyositis","volume":"88","author":"Uruha","year":"2017","journal-title":"Neurology"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1007\/s11926-021-01017-7","article-title":"Updates on the Immunopathology in Idiopathic Inflammatory Myopathies","volume":"23","author":"Uruha","year":"2021","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Franzi, S., Salajegheh, M., Nazareno, R., and Greenberg, S.A. (2013). Type 1 interferons inhibit myotube formation independently of upregulation of interferon-stimulated gene 15. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0065362"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1891","DOI":"10.1093\/brain\/aww122","article-title":"Microvascular alterations and the role of complement in dermatomyositis","volume":"139","author":"Lahoria","year":"2016","journal-title":"Brain"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"22","DOI":"10.21037\/atm.2016.12.68","article-title":"Vascular changes and perifascicular muscle fiber damage in dermatomyositis: Another question of the chicken or the egg that is on our mind","volume":"5","year":"2017","journal-title":"Ann. Transl. Med."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/0022-510X(89)90228-1","article-title":"Early ultrastructural alterations in adult dermatomyositis. Capillary abnormalities precede other structural changes in muscle","volume":"94","author":"Engel","year":"1989","journal-title":"J. Neurol. Sci."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1080\/01913120802209379","article-title":"Tubuloreticular structures in different types of myositis: Implications for pathogenesis","volume":"32","author":"Bronner","year":"2008","journal-title":"Ultrastruct. Pathol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1097\/00005072-197501000-00005","article-title":"Dermatomyositis of childhood: Ultrastructural alterations of muscle and intramuscular blood vessels","volume":"34","author":"Banker","year":"1975","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1111\/j.1600-0404.2010.01353.x","article-title":"Detection of the membrane attack complex as a diagnostic tool in dermatomyositis","volume":"123","author":"Jain","year":"2011","journal-title":"Acta Neurol. Scand."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"102993","DOI":"10.1016\/j.autrev.2021.102993","article-title":"Immune-mediated necrotizing myopathy (IMNM): A myopathological challenge","volume":"21","author":"Merlonghi","year":"2022","journal-title":"Autoimmun. Rev."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"R1173","DOI":"10.1152\/ajpregu.00735.2009","article-title":"Regulatory interactions between muscle and the immune system during muscle regeneration","volume":"298","author":"Tidball","year":"2010","journal-title":"Am. J. Physiol. Regul. Integr. Comp. Physiol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1002\/mus.24642","article-title":"The composition of cellular infiltrates in anti-HMG-CoA reductase-associated myopathy","volume":"52","author":"Chung","year":"2015","journal-title":"Muscle Nerve"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"e507","DOI":"10.1212\/WNL.0000000000004923","article-title":"Necrosis in anti-SRP(+) and anti-HMGCR(+)myopathies: Role of autoantibodies and complement","volume":"90","author":"Allenbach","year":"2018","journal-title":"Neurology"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1054\/bjoc.2001.1699","article-title":"Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: A Scottish population-based cohort study","volume":"85","author":"Stockton","year":"2001","journal-title":"Br. J. Cancer"},{"key":"ref_47","doi-asserted-by":"crossref","unstructured":"Gracia-Ramos, A.E., Martin-Nares, E., and Hernandez-Molina, G. (2021). New Onset of Autoimmune Diseases Following COVID-19 Diagnosis. Cells, 10.","DOI":"10.3390\/cells10123592"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1001\/jamaneurol.2021.2004","article-title":"Association Between SARS-CoV-2 Infection and Immune-Mediated Myopathy in Patients Who Have Died","volume":"78","author":"Aschman","year":"2021","journal-title":"JAMA Neurol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1038\/gene.2015.28","article-title":"Genome-wide association study identifies HLA 8.1 ancestral haplotype alleles as major genetic risk factors for myositis phenotypes","volume":"16","author":"Miller","year":"2015","journal-title":"Genes Immun."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1097\/BOR.0000000000000652","article-title":"Genetics of idiopathic inflammatory myopathies: Insights into disease pathogenesis","volume":"31","author":"Rothwell","year":"2019","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"996","DOI":"10.1136\/annrheumdis-2019-215046","article-title":"Focused HLA analysis in Caucasians with myositis identifies significant associations with autoantibody subgroups","volume":"78","author":"Rothwell","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/j.semarthrit.2019.03.005","article-title":"Novel susceptibility alleles in HLA region for myositis and myositis specific autoantibodies in Korean patients","volume":"49","author":"Kang","year":"2019","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1038\/nrrheum.2018.48","article-title":"Risk factors and disease mechanisms in myositis","volume":"14","author":"Miller","year":"2018","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"2905","DOI":"10.1172\/JCI118749","article-title":"Differential expression of perforin in muscle-infiltrating T cells in polymyositis and dermatomyositis","volume":"97","author":"Goebels","year":"1996","journal-title":"J. Clin. Investig."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1002\/ana.24902","article-title":"Pathogenic role of anti-signal recognition protein and anti-3-Hydroxy-3-methylglutaryl-CoA reductase antibodies in necrotizing myopathies: Myofiber atrophy and impairment of muscle regeneration in necrotizing autoimmune myopathies","volume":"81","author":"Allenbach","year":"2017","journal-title":"Ann. Neurol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1093\/rheumatology\/ker408","article-title":"Disappearance of anti-MDA-5 autoantibodies in clinically amyopathic DM\/interstitial lung disease during disease remission","volume":"51","author":"Muro","year":"2012","journal-title":"Rheumatology"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"1492","DOI":"10.1093\/rheumatology\/kex188","article-title":"Clinical characteristics and change in the antibody titres of patients with anti-MDA5 antibody-positive inflammatory myositis","volume":"56","author":"Abe","year":"2017","journal-title":"Rheumatology"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1111\/bjd.14882","article-title":"Antimelanoma differentiation-associated protein 5 antibody level is a novel tool for monitoring disease activity in rapidly progressive interstitial lung disease with dermatomyositis","volume":"176","author":"Matsushita","year":"2017","journal-title":"Br. J. Dermatol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1002\/ana.20464","article-title":"Interferon-alpha\/beta-mediated innate immune mechanisms in dermatomyositis","volume":"57","author":"Greenberg","year":"2005","journal-title":"Ann. Neurol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1111\/bpa.12772","article-title":"Sequestosome-1 (p62) expression reveals chaperone-assisted selective autophagy in immune-mediated necrotizing myopathies","volume":"30","author":"Fischer","year":"2020","journal-title":"Brain Pathol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1007\/s12016-016-8527-x","article-title":"The Immune Response and the Pathogenesis of Idiopathic Inflammatory Myositis: A Critical Review","volume":"52","author":"Ceribelli","year":"2017","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1056\/NEJMc1506827","article-title":"Inflammatory Muscle Diseases","volume":"373","author":"Dalakas","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1038\/nrrheum.2018.56","article-title":"Autoantibodies in myositis","volume":"14","author":"McHugh","year":"2018","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1111\/joim.12451","article-title":"Myositis-specific autoantibodies: An important tool to support diagnosis of myositis","volume":"280","author":"Betteridge","year":"2016","journal-title":"J. Intern. Med."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1093\/rheumatology\/kev444","article-title":"Autoantibody levels in myositis patients correlate with clinical response during B cell depletion with rituximab","volume":"55","author":"Aggarwal","year":"2016","journal-title":"Rheumatology"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1186\/s42358-018-0030-z","article-title":"Favorable rituximab response in patients with refractory idiopathic inflammatory myopathies","volume":"58","author":"Miossi","year":"2018","journal-title":"Adv. Rheumatol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1093\/rheumatology\/key450","article-title":"Efficacy and safety of rituximab in anti-synthetase antibody positive and negative subjects with idiopathic inflammatory myopathy: A registry-based study","volume":"58","author":"Leclair","year":"2019","journal-title":"Rheumatology"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1782","DOI":"10.1212\/01.wnl.0000187124.92826.20","article-title":"Plasma cells in muscle in inclusion body myositis and polymyositis","volume":"65","author":"Greenberg","year":"2005","journal-title":"Neurology"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"e451","DOI":"10.1212\/NXI.0000000000000451","article-title":"Architectural B-cell organization in skeletal muscle identifies subtypes of dermatomyositis","volume":"5","author":"Radke","year":"2018","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1007\/s00401-009-0570-8","article-title":"Inflammatory myopathy with abundant macrophages and dermatomyositis: Two stages of one disorder or two distinct entities?","volume":"118","author":"Brunn","year":"2009","journal-title":"Acta Neuropathol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1016\/0960-8966(96)00015-6","article-title":"Peripheral lymphoid tissue-like adhesion molecule expression in nodular infiltrates in inflammatory myopathies","volume":"6","author":"Engel","year":"1996","journal-title":"Neuromuscul. Disord."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1002\/eji.201343315","article-title":"The VH repertoire and clonal diversification of B cells in inflammatory myopathies","volume":"44","author":"McIntyre","year":"2014","journal-title":"Eur. J. Immunol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1186\/s13023-020-01431-7","article-title":"Factors associated with refractory autoimmune necrotizing myopathy with anti-signal recognition particle antibodies","volume":"15","author":"Zhao","year":"2020","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1186\/s13023-015-0277-y","article-title":"Inflammatory myopathy with anti-signal recognition particle antibodies: Case series of 100 patients","volume":"10","author":"Suzuki","year":"2015","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1136\/practneurol-2017-001742","article-title":"Autoantibody testing in idiopathic inflammatory myopathies","volume":"19","author":"Rietveld","year":"2019","journal-title":"Pract. Neurol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"992","DOI":"10.3389\/fimmu.2017.00992","article-title":"Dermatomyositis and Immune-Mediated Necrotizing Myopathies: A Window on Autoimmunity and Cancer","volume":"8","author":"Aussy","year":"2017","journal-title":"Front. Immunol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"2131","DOI":"10.1093\/brain\/aww054","article-title":"High risk of cancer in autoimmune necrotizing myopathies: Usefulness of myositis specific antibody","volume":"139","author":"Allenbach","year":"2016","journal-title":"Brain"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1016\/j.nmd.2019.09.014","article-title":"Malignancy and myositis, from molecular mimicry to tumor infiltrating lymphocytes","volume":"29","author":"Ros","year":"2019","journal-title":"Neuromuscul. Disord."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1084\/jem.20041367","article-title":"Enhanced autoantigen expression in regenerating muscle cells in idiopathic inflammatory myopathy","volume":"201","author":"Nagaraju","year":"2005","journal-title":"J. Exp. Med."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1056\/NEJMra1515161","article-title":"Statin-Associated Autoimmune Myopathy","volume":"374","author":"Mammen","year":"2016","journal-title":"N. Engl. J. Med."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"100013","DOI":"10.1016\/j.jtauto.2019.100013","article-title":"Frequencies and clinical associations of myositis-related antibodies in The Netherlands: A one-year survey of all Dutch patients","volume":"2","author":"Platteel","year":"2019","journal-title":"J. Transl. Autoimmun."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.jaut.2019.04.001","article-title":"Frequency, mutual exclusivity and clinical associations of myositis autoantibodies in a combined European cohort of idiopathic inflammatory myopathy patients","volume":"101","author":"Betteridge","year":"2019","journal-title":"J. Autoimmun."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1212\/WNL.0000000000002863","article-title":"Anti-TIF1-gamma antibody and cancer-associated myositis: A clinicohistopathologic study","volume":"87","author":"Hida","year":"2016","journal-title":"Neurology"},{"key":"ref_84","doi-asserted-by":"crossref","unstructured":"Mugii, N., Hasegawa, M., Matsushita, T., Hamaguchi, Y., Oohata, S., Okita, H., Yahata, T., Someya, F., Inoue, K., and Murono, S. (2016). Oropharyngeal Dysphagia in Dermatomyositis: Associations with Clinical and Laboratory Features Including Autoantibodies. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0154746"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"e70","DOI":"10.1212\/WNL.0000000000009727","article-title":"Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases","volume":"95","author":"Allenbach","year":"2020","journal-title":"Neurology"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"1839","DOI":"10.1093\/rheumatology\/keaa429","article-title":"Anti-MDA5 autoantibodies associated with juvenile dermatomyositis constitute a distinct phenotype in North America","volume":"60","author":"Mamyrova","year":"2021","journal-title":"Rheumatology"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"773352","DOI":"10.3389\/fimmu.2021.773352","article-title":"Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies","volume":"12","author":"Nombel","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.jaad.2010.09.016","article-title":"The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): A retrospective study","volume":"65","author":"Fiorentino","year":"2011","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1002\/acr.21992","article-title":"Anti-melanoma differentiation-associated protein 5-associated dermatomyositis: Expanding the clinical spectrum","volume":"65","author":"Hall","year":"2013","journal-title":"Arthritis Care Res."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1002\/art.41105","article-title":"Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy with High-Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti-Melanoma Differentiation-Associated Gene 5-Positive Dermatomyositis","volume":"72","author":"Tsuji","year":"2020","journal-title":"Arthritis Rheumatol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1007\/s12016-020-08822-5","article-title":"Interstitial Lung Disease in Anti-MDA5 Positive Dermatomyositis","volume":"60","author":"Wu","year":"2021","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1001\/archdermatol.2011.52","article-title":"Clinical correlations with dermatomyositis-specific autoantibodies in adult Japanese patients with dermatomyositis: A multicenter cross-sectional study","volume":"147","author":"Hamaguchi","year":"2011","journal-title":"Arch. Dermatol."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"151959","DOI":"10.1016\/j.semarthrit.2022.151959","article-title":"Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: A systematic review","volume":"53","author":"McPherson","year":"2022","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"1627","DOI":"10.1007\/s10067-015-2935-9","article-title":"Distinct profiles of myositis-specific autoantibodies in Chinese and Japanese patients with polymyositis\/dermatomyositis","volume":"34","author":"Chen","year":"2015","journal-title":"Clin. Rheumatol."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/j.jpeds.2010.11.033","article-title":"Anti-melanoma differentiation-associated gene 5 antibody is a diagnostic and predictive marker for interstitial lung diseases associated with juvenile dermatomyositis","volume":"158","author":"Kobayashi","year":"2011","journal-title":"J. Pediatr."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1080\/14397595.2018.1452353","article-title":"Myositis-specific autoantibodies in Japanese patients with juvenile idiopathic inflammatory myopathies","volume":"29","author":"Ueki","year":"2019","journal-title":"Mod. Rheumatol."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"1090","DOI":"10.1111\/bjd.16917","article-title":"Aberrant activation of the type I interferon system may contribute to the pathogenesis of anti-melanoma differentiation-associated gene 5 dermatomyositis","volume":"180","author":"Zhang","year":"2019","journal-title":"Br. J. Dermatol."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"85","DOI":"10.3109\/14397595.2014.900843","article-title":"Serum interferon-alpha is a useful biomarker in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis","volume":"25","author":"Horai","year":"2015","journal-title":"Mod. Rheumatol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"1276","DOI":"10.1016\/j.jid.2019.10.020","article-title":"MDA5(+) Dermatomyositis Is Associated with Stronger Skin Type I Interferon Transcriptomic Signature with Upregulation of IFN-kappa Transcript","volume":"140","author":"Cassius","year":"2020","journal-title":"J. Investig. Dermatol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1093\/intimm\/12.4.415","article-title":"In vivo cell penetration and intracellular transport of anti-Sm and anti-La autoantibodies","volume":"12","author":"Deng","year":"2000","journal-title":"Int. Immunol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1345","DOI":"10.1681\/ASN.V281345","article-title":"Murine monoclonal anti-DNA antibodies penetrate cells, bind to nuclei, and induce glomerular proliferation and proteinuria in vivo","volume":"2","author":"Vlahakos","year":"1992","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"134","DOI":"10.4172\/2155-9899.1000134","article-title":"Antibodies to an intracellular antigen penetrate neuronal cells and cause deleterious effects","volume":"4","author":"Douglas","year":"2013","journal-title":"J. Clin. Cell Immunol."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"1201","DOI":"10.1136\/annrheumdis-2020-218661","article-title":"Immune response to dermatomyositis-specific autoantigen, transcriptional intermediary factor 1gamma can result in experimental myositis","volume":"80","author":"Okiyama","year":"2021","journal-title":"Ann. Rheum. Dis."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"715","DOI":"10.2340\/00015555-1550","article-title":"TIF1gamma-overexpressing, highly progressive endometrial carcinoma in a patient with dermato-myositis positive for malignancy-associated anti-p155\/140 autoantibody","volume":"93","author":"Kasuya","year":"2013","journal-title":"Acta Derm. Venereol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1016\/j.jdermsci.2016.09.013","article-title":"Enzyme-linked immunosorbent assays for detection of anti-transcriptional intermediary factor-1 gamma and anti-Mi-2 autoantibodies in dermatomyositis","volume":"84","author":"Fujimoto","year":"2016","journal-title":"J. Dermatol. Sci."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.1002\/art.1780330908","article-title":"Antibody to signal recognition particle in polymyositis","volume":"33","author":"Targoff","year":"1990","journal-title":"Arthritis Rheum."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"2757","DOI":"10.1002\/art.27572","article-title":"A novel autoantibody recognizing 200-kd and 100-kd proteins is associated with an immune-mediated necrotizing myopathy","volume":"62","author":"Hong","year":"2010","journal-title":"Arthritis Rheum."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1038\/s41584-020-00515-9","article-title":"Immune-mediated necrotizing myopathy: Clinical features and pathogenesis","volume":"16","author":"Allenbach","year":"2020","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s11926-018-0732-6","article-title":"Immune-Mediated Necrotizing Myopathy","volume":"20","author":"Mammen","year":"2018","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"1038","DOI":"10.1136\/jnnp-2016-313166","article-title":"Clinical features and prognosis in anti-SRP and anti-HMGCR necrotising myopathy","volume":"87","author":"Watanabe","year":"2016","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1002\/acr.22920","article-title":"Longitudinal Course of Disease in a Large Cohort of Myositis Patients With Autoantibodies Recognizing the Signal Recognition Particle","volume":"69","author":"Parks","year":"2017","journal-title":"Arthritis Care Res."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1136\/annrheumdis-2016-210198","article-title":"Thigh muscle MRI in immune-mediated necrotising myopathy: Extensive oedema, early muscle damage and role of anti-SRP autoantibodies as a marker of severity","volume":"76","author":"Carrino","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"1635","DOI":"10.1136\/ard.2006.052191","article-title":"Anti-signal recognition particle autoantibodies: Marker of a necrotising myopathy","volume":"65","author":"Hengstman","year":"2006","journal-title":"Ann. Rheum. Dis."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"1961","DOI":"10.1002\/art.30344","article-title":"Correlation of anti-signal recognition particle autoantibody levels with creatine kinase activity in patients with necrotizing myopathy","volume":"63","author":"Benveniste","year":"2011","journal-title":"Arthritis Rheum."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"3219","DOI":"10.4049\/jimmunol.138.10.3219","article-title":"Characterization of human autoantibodies that selectively precipitate the 7SL RNA component of the signal recognition particle","volume":"138","author":"Okada","year":"1987","journal-title":"J. Immunol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"9507","DOI":"10.1073\/pnas.83.24.9507","article-title":"Human autoantibodies reactive with the signal-recognition particle","volume":"83","author":"Reeves","year":"1986","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"R39","DOI":"10.1186\/ar1895","article-title":"Human autoantibodies against the 54 kDa protein of the signal recognition particle block function at multiple stages","volume":"8","author":"Romisch","year":"2006","journal-title":"Arthritis Res. Ther."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/j.jneuroim.2013.08.008","article-title":"Complement-mediated muscle cell lysis: A possible mechanism of myonecrosis in anti-SRP associated necrotizing myopathy (ASANM)","volume":"264","author":"Mitrpant","year":"2013","journal-title":"J. Neuroimmunol."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1136\/annrheumdis-2018-213518","article-title":"In vivo pathogenicity of IgG from patients with anti-SRP or anti-HMGCR autoantibodies in immune-mediated necrotising myopathy","volume":"78","author":"Bergua","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1016\/j.jaut.2004.11.008","article-title":"Nuclear localizing anti-DNA antibodies enter cells via caveoli and modulate expression of caveolin and p53","volume":"24","author":"Yanase","year":"2005","journal-title":"J. Autoimmun."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1172\/JCI119517","article-title":"Receptor-mediated cellular entry of nuclear localizing anti-DNA antibodies via myosin 1","volume":"100","author":"Yanase","year":"1997","journal-title":"J. Clin. Investig."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/j.imlet.2009.04.005","article-title":"Mouse monoclonal autoantibodies penetrate mouse macrophage cells and stimulate NF-kappaB activation and TNF-alpha release","volume":"124","author":"Jang","year":"2009","journal-title":"Immunol. Lett."},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"3178","DOI":"10.1002\/eji.200838678","article-title":"Arginines in the CDR of anti-dsDNA autoantibodies facilitate cell internalization via electrostatic interactions","volume":"38","author":"Song","year":"2008","journal-title":"Eur. J. Immunol."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1038\/271067a0","article-title":"Antibody to nuclear ribonucleoprotein penetrates live human mononuclear cells through Fc receptors","volume":"271","author":"Fishbein","year":"1978","journal-title":"Nature"},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"35751","DOI":"10.1074\/jbc.M007476200","article-title":"Cell surface expression of calnexin, a molecular chaperone in the endoplasmic reticulum","volume":"275","author":"Okazaki","year":"2000","journal-title":"J. Biol. Chem."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/j.yexmp.2005.06.008","article-title":"Multinucleated giant cell formation exhibits features of phagocytosis with participation of the endoplasmic reticulum","volume":"79","author":"McNally","year":"2005","journal-title":"Exp. Mol. Pathol."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"e124","DOI":"10.1212\/NXI.0000000000000124","article-title":"Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology","volume":"2","author":"Alshehri","year":"2015","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"4087","DOI":"10.1002\/art.34673","article-title":"Antibody levels correlate with creatine kinase levels and strength in anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase-associated autoimmune myopathy","volume":"64","author":"Werner","year":"2012","journal-title":"Arthritis Rheum."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"e416","DOI":"10.1097\/MD.0000000000000416","article-title":"Statins and myotoxic effects associated with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies: An observational study in Japan","volume":"94","author":"Watanabe","year":"2015","journal-title":"Medicine"},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"2057","DOI":"10.1002\/jcb.24077","article-title":"3-hydroxy 3-methylglutaryl coenzyme A reductase inhibition impairs muscle regeneration","volume":"113","author":"Trapani","year":"2012","journal-title":"J. Cell Biochem."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1002\/jcp.21810","article-title":"3-hydroxy 3-methylglutaryl coenzyme A reductase increase is essential for rat muscle differentiation","volume":"220","author":"Martini","year":"2009","journal-title":"J. Cell Physiol."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1056\/NEJMoa0801936","article-title":"SLCO1B1 variants and statin-induced myopathy--a genomewide study","volume":"359","author":"Group","year":"2008","journal-title":"N. Engl. J. Med."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1038\/nrd4657","article-title":"Complement, a target for therapy in inflammatory and degenerative diseases","volume":"14","author":"Morgan","year":"2015","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1038\/s41582-020-0400-0","article-title":"Complement in neurological disorders and emerging complement-targeted therapeutics","volume":"16","author":"Dalakas","year":"2020","journal-title":"Nat. Rev. Neurol."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"1691","DOI":"10.3389\/fimmu.2018.01691","article-title":"Functional Characterization of Alternative and Classical Pathway C3\/C5 Convertase Activity and Inhibition Using Purified Models","volume":"9","author":"Zwarthoff","year":"2018","journal-title":"Front. Immunol."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"20160221","DOI":"10.1098\/rstb.2016.0221","article-title":"The mystery behind membrane insertion: A review of the complement membrane attack complex","volume":"372","author":"Bubeck","year":"2017","journal-title":"Philos. Trans. R. Soc. Lond. B Biol. Sci."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"2008","DOI":"10.1212\/01.WNL.0000291619.17160.b8","article-title":"Proposed immunologic models of the inflammatory myopathies and potential therapeutic implications","volume":"69","author":"Greenberg","year":"2007","journal-title":"Neurology"},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/0002-9343(75)90537-9","article-title":"Hereditary complement (C2) deficiency with dermatomyositis","volume":"58","author":"Leddy","year":"1975","journal-title":"Am. J. Med."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1016\/S0001-7310(07)70093-5","article-title":"Complement activation products (C3a and C5b-9) as markers of activity of dermatomyositis. Comparison with usual biochemical parameters","volume":"98","author":"Campo","year":"2007","journal-title":"Actas Dermosifiliogr."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"1729","DOI":"10.1172\/JCI117520","article-title":"High-dose intravenous immunoglobulin exerts its beneficial effect in patients with dermatomyositis by blocking endomysial deposition of activated complement fragments","volume":"94","author":"Basta","year":"1994","journal-title":"J. Clin. Investig."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1097\/PAI.0000000000000002","article-title":"C4d staining as immunohistochemical marker in inflammatory myopathies","volume":"22","author":"Pytel","year":"2014","journal-title":"Appl. Immunohistochem. Mol. Morphol."},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1097\/00019052-200406000-00018","article-title":"Uncertainties in the pathogenesis of adult dermatomyositis","volume":"17","author":"Greenberg","year":"2004","journal-title":"Curr. Opin. Neurol."},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1002\/ana.410270402","article-title":"Microvascular changes in early and advanced dermatomyositis: A quantitative study","volume":"27","author":"Engel","year":"1990","journal-title":"Ann. Neurol."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1056\/NEJM198602063140601","article-title":"Microvascular deposition of complement membrane attack complex in dermatomyositis","volume":"314","author":"Kissel","year":"1986","journal-title":"N. Engl. J. Med."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1056\/NEJM197202172860701","article-title":"Vascular deposits of immunoglobulin and complement in idiopathic inflammatory myopathy","volume":"286","author":"Whitaker","year":"1972","journal-title":"N. Engl. J. Med."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/j.nmd.2016.02.014","article-title":"C5b-9 deposits on endomysial capillaries in non-dermatomyositis cases","volume":"26","author":"Braczynski","year":"2016","journal-title":"Neuromuscul. Disord."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"1080","DOI":"10.1046\/j.1365-2133.2001.04204.x","article-title":"Hereditary complement (C9) deficiency associated with dermatomyositis","volume":"144","author":"Ichikawa","year":"2001","journal-title":"Br. J. Dermatol."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"e558","DOI":"10.1212\/NXI.0000000000000558","article-title":"PD1 pathway in immune-mediated myopathies: Pathogenesis of dysfunctional T cells revisited","volume":"6","author":"Knauss","year":"2019","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"917","DOI":"10.3389\/fimmu.2020.00917","article-title":"Complement Inhibitor Therapy for Myasthenia Gravis","volume":"11","author":"Albazli","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1038\/nri3581","article-title":"Regulation of type I interferon responses","volume":"14","author":"Ivashkiv","year":"2014","journal-title":"Nat. Rev. Immunol."},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1146\/annurev-med-051113-024537","article-title":"The JAK-STAT pathway: Impact on human disease and therapeutic intervention","volume":"66","author":"Schwartz","year":"2015","journal-title":"Annu. Rev. Med."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"3784","DOI":"10.1002\/art.22928","article-title":"Type I interferon-inducible gene expression in blood is present and reflects disease activity in dermatomyositis and polymyositis","volume":"56","author":"Walsh","year":"2007","journal-title":"Arthritis Rheum."},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"59","DOI":"10.2119\/2006-00085.Baechler","article-title":"An interferon signature in the peripheral blood of dermatomyositis patients is associated with disease activity","volume":"13","author":"Baechler","year":"2007","journal-title":"Mol. Med."},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"1835","DOI":"10.1126\/science.284.5421.1835","article-title":"The nature of the principal type 1 interferon-producing cells in human blood","volume":"284","author":"Siegal","year":"1999","journal-title":"Science"},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1038\/icb.2012.10","article-title":"Role of type I interferons in the activation of autoreactive B cells","volume":"90","author":"Kiefer","year":"2012","journal-title":"Immunol. Cell Biol."},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1016\/j.autrev.2017.07.021","article-title":"Using multi-omics methods to understand dermatomyositis\/polymyositis","volume":"16","author":"Gao","year":"2017","journal-title":"Autoimmun Rev."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1002\/ana.21805","article-title":"Interferon-stimulated gene 15 (ISG15) conjugates proteins in dermatomyositis muscle with perifascicular atrophy","volume":"67","author":"Salajegheh","year":"2010","journal-title":"Ann. Neurol"},{"key":"ref_158","first-page":"e1193","article-title":"Identification of distinctive interferon gene signatures in different types of myositis","volume":"93","author":"Derfoul","year":"2019","journal-title":"Neurology"},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1007\/s00401-021-02365-5","article-title":"A network of core and subtype-specific gene expression programs in myositis","volume":"142","author":"Amici","year":"2021","journal-title":"Acta Neuropathol."},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1136\/ard.2010.139949","article-title":"Interferon beta is associated with type 1 interferon-inducible gene expression in dermatomyositis","volume":"70","author":"Liao","year":"2011","journal-title":"Ann. Rheum. Dis."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"1224","DOI":"10.1111\/bjd.15006","article-title":"Correlation of cutaneous disease activity with type 1 interferon gene signature and interferon beta in dermatomyositis","volume":"176","author":"Huard","year":"2017","journal-title":"Br. J. Dermatol."},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1038\/nrrheum.2018.42","article-title":"Treatment in myositis","volume":"14","author":"Oddis","year":"2018","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_163","first-page":"CD003643","article-title":"Immunosuppressant and immunomodulatory treatment for dermatomyositis and polymyositis","volume":"2012","author":"Gordon","year":"2012","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"1993","DOI":"10.1056\/NEJM199312303292704","article-title":"A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis","volume":"329","author":"Dalakas","year":"1993","journal-title":"N. Engl. J. Med."},{"key":"ref_165","doi-asserted-by":"crossref","first-page":"e23677","DOI":"10.1097\/MD.0000000000023677","article-title":"Prospective, double-blind, randomized, placebo-controlled phase III study evaluating efficacy and safety of octagam 10% in patients with dermatomyositis (\u201cProDERM Study\u201d)","volume":"100","author":"Aggarwal","year":"2021","journal-title":"Medicine"},{"key":"ref_166","doi-asserted-by":"crossref","first-page":"2089","DOI":"10.1007\/s10067-015-3059-y","article-title":"The evidence for immunotherapy in dermatomyositis and polymyositis: A systematic review","volume":"34","author":"Vermaak","year":"2015","journal-title":"Clin. Rheumatol."},{"key":"ref_167","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1016\/j.nmd.2010.03.011","article-title":"Oral dexamethasone pulse therapy versus daily prednisolone in sub-acute onset myositis, a randomised clinical trial","volume":"20","author":"Hoogendijk","year":"2010","journal-title":"Neuromuscul. Disord."},{"key":"ref_168","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1097\/00124743-199504000-00007","article-title":"The Use of Low-dose Oral Methotrexate in the Treatment of Polymyositis and Dermatomyositis","volume":"1","author":"Newman","year":"1995","journal-title":"J. Clin. Rheumatol."},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1093\/rheumatology\/keh499","article-title":"Mycophenolate mofetil (CellCept): An alternative therapy for autoimmune inflammatory myopathy","volume":"44","author":"Majithia","year":"2005","journal-title":"Rheumatology"},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1093\/rheumatology\/kel336","article-title":"Mycophenolate mofetil treatment in resistant myositis","volume":"46","author":"Pisoni","year":"2007","journal-title":"Rheumatology"},{"key":"ref_171","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.autrev.2009.04.003","article-title":"Intravenous immunoglobulin as add on treatment with mycophenolate mofetil in severe myositis","volume":"9","author":"Danieli","year":"2009","journal-title":"Autoimmun. Rev."},{"key":"ref_172","doi-asserted-by":"crossref","first-page":"102997","DOI":"10.1016\/j.autrev.2021.102997","article-title":"Efficacy and safety of intravenous and subcutaneous immunoglobulin therapy in idiopathic inflammatory myopathy: A systematic review and meta-analysis","volume":"21","author":"Goswami","year":"2022","journal-title":"Autoimmun. Rev."},{"key":"ref_173","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1002\/art.38910","article-title":"Review: Intravenous immunoglobulin and B cells: When the product regulates the producer","volume":"67","author":"Seite","year":"2015","journal-title":"Arthritis Rheumatol."},{"key":"ref_174","doi-asserted-by":"crossref","first-page":"1150","DOI":"10.1111\/jdv.12223","article-title":"Clinical efficacy of intravenous immunoglobulins for the treatment of dermatomyositis skin lesions without muscle disease","volume":"28","author":"Bounfour","year":"2014","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_175","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1016\/j.jaad.2013.06.007","article-title":"Intravenous immunoglobulin for refractory cutaneous dermatomyositis: A retrospective analysis from an academic medical center","volume":"69","author":"Femia","year":"2013","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_176","doi-asserted-by":"crossref","first-page":"1680","DOI":"10.1056\/NEJMc1506163","article-title":"Intravenous Immune Globulin for Statin-Triggered Autoimmune Myopathy","volume":"373","author":"Mammen","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_177","doi-asserted-by":"crossref","first-page":"996","DOI":"10.1001\/jamaneurol.2015.1207","article-title":"Clinical Features and Treatment Outcomes of Necrotizing Autoimmune Myopathy","volume":"72","author":"Kassardjian","year":"2015","journal-title":"JAMA Neurol."},{"key":"ref_178","doi-asserted-by":"crossref","first-page":"1784","DOI":"10.1093\/rheumatology\/keaa459","article-title":"Intravenous immunoglobulins as first-line treatment in idiopathic inflammatory myopathies: A pilot study","volume":"60","author":"Lim","year":"2021","journal-title":"Rheumatology"},{"key":"ref_179","doi-asserted-by":"crossref","first-page":"844","DOI":"10.1136\/annrheumdis-2012-201612","article-title":"Induction therapy with adalimumab plus methotrexate for 24 weeks followed by methotrexate monotherapy up to week 48 versus methotrexate therapy alone for DMARD-naive patients with early rheumatoid arthritis: HIT HARD, an investigator-initiated study","volume":"72","author":"Detert","year":"2013","journal-title":"Ann. Rheum. Dis."},{"key":"ref_180","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1111\/bjh.12629","article-title":"The mechanisms of action of plasma exchange","volume":"164","author":"Reeves","year":"2014","journal-title":"Br. J. Haematol."},{"key":"ref_181","doi-asserted-by":"crossref","first-page":"1380","DOI":"10.1056\/NEJM199205213262102","article-title":"Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis","volume":"326","author":"Miller","year":"1992","journal-title":"N. Engl. J. Med."},{"key":"ref_182","doi-asserted-by":"crossref","first-page":"3284","DOI":"10.1093\/rheumatology\/keaa123","article-title":"Efficacy of plasma exchange in anti-MDA5-positive dermatomyositis with interstitial lung disease under combined immunosuppressive treatment","volume":"59","author":"Shirakashi","year":"2020","journal-title":"Rheumatology"},{"key":"ref_183","doi-asserted-by":"crossref","first-page":"326","DOI":"10.3389\/fmed.2019.00326","article-title":"Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment","volume":"6","author":"Hervier","year":"2019","journal-title":"Front. Med."},{"key":"ref_184","doi-asserted-by":"crossref","first-page":"2113","DOI":"10.1007\/s10067-018-4174-3","article-title":"Oral cyclophosphamide in treatment of patients with refractory idiopathic inflammatory myopathies: A retrospective observational study","volume":"37","author":"Bae","year":"2018","journal-title":"Clin. Rheumatol."},{"key":"ref_185","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1097\/BOR.0000000000000109","article-title":"Therapy of myositis: Biological and physical","volume":"26","author":"Lundberg","year":"2014","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_186","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1097\/BOR.0000000000000432","article-title":"Biologics for idiopathic inflammatory myopathies","volume":"29","author":"Aggarwal","year":"2017","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_187","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1002\/art.37754","article-title":"Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: A randomized, placebo-phase trial","volume":"65","author":"Oddis","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_188","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1002\/art.38270","article-title":"Predictors of clinical improvement in rituximab-treated refractory adult and juvenile dermatomyositis and adult polymyositis","volume":"66","author":"Aggarwal","year":"2014","journal-title":"Arthritis Rheumatol."},{"key":"ref_189","doi-asserted-by":"crossref","first-page":"820163","DOI":"10.3389\/fimmu.2021.820163","article-title":"Rituximab in the Treatment of Interstitial Lung Diseases Related to Anti-Melanoma Differentiation-Associated Gene 5 Dermatomyositis: A Systematic Review","volume":"12","author":"He","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_190","doi-asserted-by":"crossref","first-page":"2673","DOI":"10.1111\/all.14704","article-title":"Systematic review of safety and efficacy of belimumab in treating immune-mediated disorders","volume":"76","author":"Kaegi","year":"2021","journal-title":"Allergy"},{"key":"ref_191","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1136\/annrheumdis-2012-202794","article-title":"A phase 1b clinical trial evaluating sifalimumab, an anti-IFN-alpha monoclonal antibody, shows target neutralisation of a type I IFN signature in blood of dermatomyositis and polymyositis patients","volume":"73","author":"Higgs","year":"2014","journal-title":"Ann. Rheum. Dis."},{"key":"ref_192","doi-asserted-by":"crossref","first-page":"1609","DOI":"10.1093\/brain\/awy105","article-title":"JAK inhibitor improves type I interferon induced damage: Proof of concept in dermatomyositis","volume":"141","author":"Ladislau","year":"2018","journal-title":"Brain"},{"key":"ref_193","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1002\/art.41602","article-title":"Study of Tofacitinib in Refractory Dermatomyositis: An Open-Label Pilot Study of Ten Patients","volume":"73","author":"Paik","year":"2021","journal-title":"Arthritis Rheumatol."},{"key":"ref_194","doi-asserted-by":"crossref","first-page":"1710","DOI":"10.3899\/jrheum.180060","article-title":"Complement C5-blocking Agent in Refractory Dermatomyositis","volume":"45","author":"Faguer","year":"2018","journal-title":"J. Rheumatol."},{"key":"ref_195","first-page":"S489","article-title":"A pilot study of eculizumab in patients with dermatomyositis","volume":"46","author":"Takada","year":"2002","journal-title":"Arthritis Rheum."},{"key":"ref_196","doi-asserted-by":"crossref","first-page":"799","DOI":"10.1016\/S0889-857X(02)00025-X","article-title":"Possible pathogenic mechanisms in inflammatory myopathies","volume":"28","author":"Lundberg","year":"2002","journal-title":"Rheum. Dis. Clin. N. Am."},{"key":"ref_197","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1016\/j.semarthrit.2006.07.003","article-title":"Tumor necrosis factor-alpha in inflammatory myopathies: Pathophysiology and therapeutic implications","volume":"36","author":"Efthimiou","year":"2006","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_198","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1136\/ard.2005.048744","article-title":"Possible role for tumour necrosis factor inhibitors in the treatment of resistant dermatomyositis and polymyositis: A retrospective study of eight patients","volume":"65","author":"Efthimiou","year":"2006","journal-title":"Ann. Rheum. Dis."},{"key":"ref_199","doi-asserted-by":"crossref","first-page":"1670","DOI":"10.1136\/ard.2007.077974","article-title":"A high incidence of disease flares in an open pilot study of infliximab in patients with refractory inflammatory myopathies","volume":"67","author":"Dastmalchi","year":"2008","journal-title":"Ann. Rheum. Dis."},{"key":"ref_200","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1016\/j.semarthrit.2017.10.010","article-title":"A randomized, double-blind, placebo-controlled trial of infliximab in refractory polymyositis and dermatomyositis","volume":"47","author":"Schiffenbauer","year":"2018","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_201","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1002\/ana.22477","article-title":"A randomized, pilot trial of etanercept in dermatomyositis","volume":"70","year":"2011","journal-title":"Ann. Neurol."},{"key":"ref_202","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1001\/archdermatol.2010.142","article-title":"Tumor necrosis factor inhibitor-associated dermatomyositis","volume":"146","author":"Klein","year":"2010","journal-title":"Arch. Dermatol."},{"key":"ref_203","first-page":"192","article-title":"Anti-tumor necrosis factor inhibitor therapy-induced dermatomyositis and fasciitis","volume":"39","author":"Riolo","year":"2012","journal-title":"J. Rheumatol."},{"key":"ref_204","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1046\/j.1365-2990.1998.00092.x","article-title":"Local expression of cytokines in idiopathic inflammatory myopathies","volume":"24","author":"Lepidi","year":"1998","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_205","first-page":"958","article-title":"Tocilizumab in Myositis: Results of a Phase IIb Double-Blind Randomized Controlled Trial","volume":"72","author":"Aggarwal","year":"2020","journal-title":"Arthritis Rheumatol."},{"key":"ref_206","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1002\/art.22388","article-title":"Immunolocalization of interleukin-1 receptors in the sarcolemma and nuclei of skeletal muscle in patients with idiopathic inflammatory myopathies","volume":"56","author":"Grundtman","year":"2007","journal-title":"Arthritis Rheum."},{"key":"ref_207","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1136\/annrheumdis-2012-202857","article-title":"Anakinra treatment in patients with refractory inflammatory myopathies and possible predictive response biomarkers: A mechanistic study with 12 months follow-up","volume":"73","author":"Zong","year":"2014","journal-title":"Ann. Rheum. Dis."},{"key":"ref_208","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1016\/j.jaut.2019.04.016","article-title":"Abatacept modulates CD80 and CD86 expression and memory formation in human B-cells","volume":"101","author":"Lorenzetti","year":"2019","journal-title":"J. Autoimmun."},{"key":"ref_209","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1136\/annrheumdis-2017-211751","article-title":"Abatacept in the treatment of adult dermatomyositis and polymyositis: A randomised, phase IIb treatment delayed-start trial","volume":"77","author":"Tjarnlund","year":"2018","journal-title":"Ann. Rheum. Dis."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/8\/4301\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:53:28Z","timestamp":1760136808000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/8\/4301"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,13]]},"references-count":209,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2022,4]]}},"alternative-id":["ijms23084301"],"URL":"https:\/\/doi.org\/10.3390\/ijms23084301","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,13]]}}}