{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T15:28:50Z","timestamp":1791300530408,"version":"4.1.0"},"reference-count":34,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2017,5,18]],"date-time":"2017-05-18T00:00:00Z","timestamp":1495065600000},"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 (IIMs) are a group of autoimmune muscle diseases with significant morbidity and mortality. This review details and updates the pathogenesis and emerging importance of myositis-specific antibodies in the development of IIMs. An increase in the understanding of how these myositis-specific antibodies play a role in IIMs has led to the further categorization of IIMs from the traditional polymyositis versus dermatomyositis, to additional subcategories of IIMs such as necrotizing autoimmune myositis (NAM). The diagnosis of IIMs, including manual muscle testing, laboratory studies, and non-invasive imaging have become important in classifying IIM subtypes and for identifying disease severity. Treatment has evolved from an era where glucocorticoid therapy was the only option to a time now that includes traditional steroid-sparing agents along with immunoglobulin therapy and biologics, such as rituximab.<\/jats:p>","DOI":"10.3390\/ijms18051084","type":"journal-article","created":{"date-parts":[[2017,5,23]],"date-time":"2017-05-23T01:47:33Z","timestamp":1495504053000},"page":"1084","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":48,"title":["Idiopathic Inflammatory Myopathies: A Review of the Classification and Impact of Pathogenesis"],"prefix":"10.3390","volume":"18","author":[{"given":"Dana","family":"Mandel","sequence":"first","affiliation":[{"name":"Department of Medicine, Division of Rheumatic Diseases, Case Western Reserve University School of Medicine and University Hospitals Cleveland Medical Center, Cleveland, OH 44106-5076, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles","family":"Malemud","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatic Diseases, Case Western Reserve University School of Medicine and University Hospitals Cleveland Medical Center, Cleveland, OH 44106-5076, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ali","family":"Askari","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Rheumatic Diseases, Case Western Reserve University School of Medicine and University Hospitals Cleveland Medical Center, Cleveland, OH 44106-5076, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2017,5,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1002\/mus.24566","article-title":"An overview of polymyositis and dermatomyositis","volume":"51","author":"Findlay","year":"2015","journal-title":"Muscle Nerve"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"R22","DOI":"10.1186\/ar3704","article-title":"Predictors of survival in a cohort of patients with polymyositis and dermatomyositis: Effect of corticosteroids, methotrexate and azathioprine","volume":"14","author":"Schiopu","year":"2012","journal-title":"Arthritis Res. Ther."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"234","DOI":"10.3389\/fimmu.2016.00234","article-title":"Autoimmune myopathies: Where do we stand?","volume":"7","author":"Simon","year":"2016","journal-title":"Front. Immunol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1734","DOI":"10.1056\/NEJMra1402225","article-title":"Inflammatory muscle diseases","volume":"372","author":"Dalakas","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1111\/joim.12524","article-title":"Diagnosis and classification of idiopathic inflammatory myopathies","volume":"280","author":"Lundberg","year":"2016","journal-title":"J. Intern. Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1016\/j.nmd.2014.12.001","article-title":"205th ENMC International Workshop: Pathology diagnosis of idiopathic inflammatory myopathies Part II 28\u201330 March 2014, Naarden, The Netherlands","volume":"25","author":"Aronica","year":"2015","journal-title":"Neuromuscul. Disord."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1265","DOI":"10.1586\/1744666X.2015.1082908","article-title":"Treatment of inflammatory myopathy: Emerging therapies and therapeutic targets","volume":"11","author":"Aggarwal","year":"2015","journal-title":"Expert Rev. Clin. Immunol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1111\/joim.12515","article-title":"New ways to subclassify patients with myositis","volume":"280","author":"Lundberg","year":"2016","journal-title":"J. Intern. Med."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Day, J., Patel, S., and Limaye, V. (2016). The role of magnetic resonance imaging techniques in evaluation and management of the idiopathic inflammatory myopathies. Semin. Arthritis Rheum.","DOI":"10.1016\/j.semarthrit.2016.11.001"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1016\/0049-0172(82)90061-0","article-title":"Cardiac abnormalities in polymyositis\/dermatomyositis","volume":"12","author":"Askari","year":"1982","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1056\/NEJM197502132920706","article-title":"Polymyositis and Dermatomyositis (first of two parts)","volume":"292","author":"Bohan","year":"1975","journal-title":"N. Engl. J. Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1056\/NEJM197502202920807","article-title":"Polymyositis and Dermatomyositis (second of two parts)","volume":"292","author":"Bohan","year":"1975","journal-title":"N. Engl. J. Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/S0140-6736(03)14368-1","article-title":"Polymyositis and dermatomyositis","volume":"362","author":"Dalakas","year":"2003","journal-title":"Lancet"},{"key":"ref_14","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, 10\u201312 October 2003, Naarden, The Netherlands","volume":"14","author":"Hoogendijk","year":"2004","journal-title":"Neuromuscul. Disord."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1097\/BOR.0000000000000328","article-title":"New developments in genetics of myositis","volume":"28","author":"Rothwell","year":"2016","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"3784","DOI":"10.1002\/art.22928","article-title":"Type I interferon\u2013inducible 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_17","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1038\/gene.2011.61","article-title":"Relationship between disease activity and type 1 interferon- and other cytokine-inducible gene expression in blood in dermatomyositis and polymyositis","volume":"13","author":"Greenberg","year":"2011","journal-title":"Genes Immun."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s12016-015-8510-y","article-title":"A comprehensive overview on myositis-specific antibodies: New and old biomarkers in idiopathic inflammatory myopathy","volume":"52","author":"Satoh","year":"2017","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/j.autrev.2012.01.006","article-title":"Comparison of long-term outcome between anti-Jo-1 and anti-PL7\/PL12 positive patients with antisythetase syndrome","volume":"11","author":"Marie","year":"2012","journal-title":"Autoimmun. Rev."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1007\/s00408-014-9665-7","article-title":"Comparison of pulmonary involvement between patients expressing anti-PL-7 and anti-Jo-1 antibodies","volume":"193","author":"Tomonaga","year":"2015","journal-title":"Lung"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1097\/MD.0b013e318260977c","article-title":"Anti-PL-7 (anti-threonyl-tRNA synthase) antisynthase syndrome: Clinical manifestations in a series of patients from a European multicenter study (EUMYONET) and review of the literature","volume":"91","author":"Vincze","year":"2012","journal-title":"Medicine"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1093\/rheumatology\/ket279","article-title":"Immune mechanisms in polymyositis and dermatomyositis and potential targets for therapy","volume":"53","author":"Venalis","year":"2014","journal-title":"Rheumatology"},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Pandya, J.M., Loell, I., Hossain, M.S., Zong, M., Alexanderson, H., Raghavan, S., Lundberg, I.E., and Malmstr\u00f6m, V. (2016). Effects of conventional immunosuppressive treatment of CD244+ (CD28null) and FOXP3+ T cells in the inflamed muscles of patients with polymyositis and dermatomyositis. Arthritis Res. Ther., 18.","DOI":"10.1186\/s13075-016-0974-5"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1111\/joim.12511","article-title":"Update on the pharmacological treatment of adult myositis","volume":"280","author":"Oddis","year":"2016","journal-title":"J. Intern. Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1136\/ard.61.8.718","article-title":"Standardised nomenclature for glucocorticoid dosages and glucocorticoid treatment regimens: Current questions and tentative answers in rheumatology","volume":"61","author":"Buttgereit","year":"2002","journal-title":"Ann. Rheum. Dis."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1038\/nature09102","article-title":"TLR recognition of self-nucleic acids hampers glucocorticoid activity in lupus","volume":"465","author":"Guiducci","year":"2010","journal-title":"Nature"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1016\/0002-9343(76)90327-2","article-title":"Steroid myopathy in connective tissue disease","volume":"61","author":"Askari","year":"1976","journal-title":"Am. J. Med."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"18683","DOI":"10.3390\/ijms160818683","article-title":"Scanning for Therapeutic Targets within the Cytokine Network of Idiopathic Inflammatory Myopathies","volume":"16","year":"2015","journal-title":"Int. J. Mol. Sci."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1016\/j.autrev.2015.12.003","article-title":"Long-term subcutaneous immunoglobulin use in inflammatory myopathies: A retrospective review of 19 cases","volume":"15","author":"Cherin","year":"2016","journal-title":"Autoimmun. Rev."},{"key":"ref_30","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_31","doi-asserted-by":"crossref","first-page":"944","DOI":"10.1001\/jamadermatol.2016.0866","article-title":"Tofacitinib citrate for refractory cutaneous dermatomyositis","volume":"152","author":"Kurtzman","year":"2016","journal-title":"JAMA Dermatol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"817.3","DOI":"10.1136\/annrheumdis-2015-eular.4518","article-title":"Poster Presentation: SAT0436\u2005Abatacept in the Treatment of Adult Dermatomyositis and Polymyositis: Artemis, a Randomized, Treatment Delayed-Start Trial","volume":"74","author":"Dastmalchi","year":"2015","journal-title":"Ann. Rheum. Dis."},{"key":"ref_33","doi-asserted-by":"crossref","unstructured":"Rasheed, K., Sethi, P., and Bixby, E. (2013). Severe vitamin D deficiency induced myopathy associated with rhabydomyolysis. N. Am. J. Med. Sci., 5.","DOI":"10.4103\/1947-2714.112491"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"876319","DOI":"10.1155\/2013\/876319","article-title":"Vitamin D Receptor Agonists Target CXCL10: New Therapeutic Tools for Resolution of Inflammation","volume":"2013","author":"Scolletta","year":"2013","journal-title":"Mediators Inflamm."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/18\/5\/1084\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T18:36:12Z","timestamp":1760207772000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/18\/5\/1084"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,5,18]]},"references-count":34,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2017,5]]}},"alternative-id":["ijms18051084"],"URL":"https:\/\/doi.org\/10.3390\/ijms18051084","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,5,18]]}}}