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Lupus 25: 925-933, 2016.","DOI":"10.1177\/0961203316651748"},{"key":"2","doi-asserted-by":"crossref","unstructured":"2. Tsuji H, Nakashima R, Mimori T. Perspectives in the treatment of interstitial lung disease accompanied with anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Int J Rheum Dis 27: e15201, 2024.","DOI":"10.1111\/1756-185X.15201"},{"key":"3","doi-asserted-by":"crossref","unstructured":"3. Tsuji H, Nakashima R, Yasumi T, et al. Differences in the autoantibody phenotypes and long-term outcomes between juvenile- and adult-idiopathic inflammatory myopathies. Semin Arthritis Rheum 68: 152530, 2024.","DOI":"10.1016\/j.semarthrit.2024.152530"},{"key":"4","doi-asserted-by":"crossref","unstructured":"4. Nakashima R, Imura Y, Kobayashi S, et al. The RIG-I-like receptor IFIH1\/MDA5 is a dermatomyositis-specific autoantigen identified by the anti-CADM-140 antibody. Rheumatology (Oxford) 49: 433-440, 2010.","DOI":"10.1093\/rheumatology\/kep375"},{"key":"5","doi-asserted-by":"crossref","unstructured":"5. Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R. Anti-melanoma differentiation-associated gene 5 is associated with rapidly progressive lung disease and poor survival in US patients with amyopathic and myopathic dermatomyositis. Arthritis Care Res (Hoboken) 68: 689-694, 2016.","DOI":"10.1002\/acr.22728"},{"key":"6","doi-asserted-by":"crossref","unstructured":"6. Sato S, Hoshino K, Satoh T, et al. RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: association with rapidly progressive interstitial lung disease. Arthritis Rheum 60: 2193-2200, 2009.","DOI":"10.1002\/art.24621"},{"key":"7","doi-asserted-by":"crossref","unstructured":"7. Sato S, Hirakata M, Kuwana M, et al. Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. Arthritis Rheum 52: 1571-1576, 2005.","DOI":"10.1002\/art.21023"},{"key":"8","doi-asserted-by":"crossref","unstructured":"8. Sato S, Masui K, Nishina N, et al.; JAMI investigators. Initial predictors of poor survival in myositis-associated interstitial lung disease: a multicentre cohort of 497 patients. Rheumatology (Oxford) 57: 1212-1221, 2018.","DOI":"10.1093\/rheumatology\/key060"},{"key":"9","doi-asserted-by":"crossref","unstructured":"9. Sato S, Murakami A, Kuwajima A, et al. Clinical utility of an enzyme-linked immunosorbent assay for detecting anti-melanoma differentiation-associated gene 5 autoantibodies. PLoS One 11: e0154285, 2016.","DOI":"10.1371\/journal.pone.0154285"},{"key":"10","doi-asserted-by":"crossref","unstructured":"10. So H, So J, Lam TT, et al. Performance of the 2017 European Alliance of Associations for Rheumatology\/American College of Rheumatology classification criteria in patients with idiopathic inflammatory myopathy and anti-melanoma differentiation-associated protein 5 positivity. Arthritis Rheumatol 74: 1588-1592, 2022.","DOI":"10.1002\/art.42150"},{"key":"11","doi-asserted-by":"crossref","unstructured":"11. Romero-Bueno F, Diaz Del Campo P, Trallero-Aragu\u00e1s E, et al.; the MEDRAS (Spanish MDA5 Register) group. Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease. Semin Arthritis Rheum 50: 776-790, 2020.","DOI":"10.1016\/j.semarthrit.2020.03.007"},{"key":"12","doi-asserted-by":"crossref","unstructured":"12. Chen Z, Wang X, Ye S. Tofacitinib in amyopathic dermatomyositis-associated interstitial lung disease. N Engl J Med 381: 291-293, 2019.","DOI":"10.1056\/NEJMc1900045"},{"key":"13","doi-asserted-by":"crossref","unstructured":"13. Shirakashi M, Nakashima R, Tsuji H, et al. Efficacy of plasma exchange in anti-MDA5-positive dermatomyositis with interstitial lung disease under combined immunosuppressive treatment. Rheumatology (Oxford) 59: 3284-3292, 2020.","DOI":"10.1093\/rheumatology\/keaa123"},{"key":"14","doi-asserted-by":"crossref","unstructured":"14. Christensen ML, Pachman LM, Schneiderman R, Patel DC, Friedman JM. Prevalence of coxsackie B virus antibodies in patients with juvenile dermatomyositis. Arthritis Rheum 29: 1365-1370, 1986.","DOI":"10.1002\/art.1780291109"},{"key":"15","doi-asserted-by":"publisher","unstructured":"15. Nishina N, Sato S, Masui K, Gono T, Kuwana M. Seasonal and residential clustering at disease onset of anti-MDA5-associated interstitial lung disease. RMD Open 6: e001202, 2020.","DOI":"10.1136\/rmdopen-2020-001202"},{"key":"16","doi-asserted-by":"crossref","unstructured":"16. Gono T, Kawaguchi Y, Kuwana M, et al. Brief Report: association of HLA-DRB1*0101\/*0405 with susceptibility to anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis in the Japanese population. Arthritis Rheum 64: 3736-3740, 2012.","DOI":"10.1002\/art.34657"},{"key":"17","doi-asserted-by":"crossref","unstructured":"17. Chen Z, Wang Y, Kuwana M, et al. HLA-DRB1 alleles as genetic risk factors for the development of anti-MDA5 antibodies in patients with dermatomyositis. J Rheumatol 44: 1389-1393, 2017.","DOI":"10.3899\/jrheum.170165"},{"key":"18","doi-asserted-by":"crossref","unstructured":"18. Yang X, Yu C, Zhang X, et al. The Prospective Registry of MyositIS (PROMIS): I. Next-generation sequencing identifies <i>HLA-DQA1<\/i> as a novel genetic risk of anti-MDA5 antibody-positive dermatomyositis. Ann Rheum Dis 84: 1221-1230, 2025.","DOI":"10.1016\/j.ard.2025.02.017"},{"key":"19","doi-asserted-by":"crossref","unstructured":"19. Muro Y, Sugiura K, Hoshino K, Akiyama M, Tamakoshi K. Epidemiologic study of clinically amyopathic dermatomyositis and anti-melanoma differentiation-associated gene 5 antibodies in central Japan. Arthritis Res Ther 13: R214, 2011.","DOI":"10.1186\/ar3547"},{"key":"20","doi-asserted-by":"crossref","unstructured":"20. Palterer B, Mazzoni A, Infantino M, et al. Seasonal patterns of myositis-specific and myositis-associated autoantibodies in Italy: seasonal patterns of myositis autoantibodies. Immunol Lett 272: 106966, 2025.","DOI":"10.1016\/j.imlet.2024.106966"},{"key":"21","doi-asserted-by":"crossref","unstructured":"21. Jayaraman S, Tiniakou E, Morgenlander WR, Na M, Christopher-Stine L, Larman HB. Comprehensive enteroviral serology links infection and anti-melanoma differentiation-associated protein 5 dermatomyositis. ACR Open Rheumatol 7: e11752, 2025.","DOI":"10.1002\/acr2.11752"},{"key":"22","doi-asserted-by":"crossref","unstructured":"22. Liu Y, Feng S, Liu X, et al. <i>IFN-beta<\/i> and <i>EIF2AK2<\/i> are potential biomarkers for interstitial lung disease in anti-MDA5 positive dermatomyositis. Rheumatology (Oxford) 62: 3724-3731, 2023.","DOI":"10.1093\/rheumatology\/kead117"},{"key":"23","doi-asserted-by":"crossref","unstructured":"23. Kochi Y, Kamatani Y, Kondo Y, et al. Splicing variant of <i>WDFY4<\/i> augments MDA5 signalling and the risk of clinically amyopathic dermatomyositis. Ann Rheum Dis 77: 602-611, 2018.","DOI":"10.1136\/annrheumdis-2017-212149"},{"key":"24","doi-asserted-by":"crossref","unstructured":"24. Guo L, Zhang X, Pu W, et al. <i>WDFY4<\/i> polymorphisms in Chinese patients with anti-MDA5 dermatomyositis is associated with rapid progressive interstitial lung disease. Rheumatology (Oxford) 62: 2320-2324, 2023.","DOI":"10.1093\/rheumatology\/kead006"},{"key":"25","doi-asserted-by":"crossref","unstructured":"25. Gono T, Miyake K, Kawaguchi Y, Kaneko H, Shinozaki M, Yamanaka H. Hyperferritinaemia and macrophage activation in a patient with interstitial lung disease with clinically amyopathic DM. Rheumatology (Oxford) 51: 1336-1338, 2012.","DOI":"10.1093\/rheumatology\/kes012"},{"key":"26","unstructured":"26. Ravelli A, Minoia F, Dav\u00ec S, et al.; the Paediatric Rheumatology International Trials Organisation, the Childhood Arthritis and Rheumatology Research Alliance, the Pediatric Rheumatology Cllaborative Study Group, the Histiocyte Society. 2016 Classification criteria for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a European League Against Rheumatism\/American College of Rheumatology\/Paediatric Rheumatology International Trials Organisation collaborative initiative. Arthritis Rheumatol 68: 566-576, 2016."},{"key":"27","doi-asserted-by":"crossref","unstructured":"27. Fattorini F, Conticini E, Dourado E, et al. Idiopathic inflammatory myopathies: one year in review 2024. Clin Exp Rheumatol 43: 167-177, 2025.","DOI":"10.55563\/clinexprheumatol\/yizkja"},{"key":"28","doi-asserted-by":"crossref","unstructured":"28. Gono T, Sato S, Kawaguchi Y, et al. Anti-MDA5 antibody, ferritin and IL-18 are useful for the evaluation of response to treatment in interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis. Rheumatology (Oxford) 51: 1563-1570, 2012.","DOI":"10.1093\/rheumatology\/kes102"},{"key":"29","doi-asserted-by":"crossref","unstructured":"29. Nara M, Komatsuda A, Omokawa A, et al. Serum interleukin 6 levels as a useful prognostic predictor of clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease. Mod Rheumatol 24: 633-636, 2014.","DOI":"10.3109\/14397595.2013.844390"},{"key":"30","doi-asserted-by":"crossref","unstructured":"30. Kuzumi A, Fukasawa T, Yamashita T, et al. Serum interleukin-34 levels in dermatomyositis: a potential biomarker for anti-MDA5-antibody-associated interstitial lung disease. Rheumatology (Oxford) keae 313, 2024.","DOI":"10.1093\/rheumatology\/keae313"},{"key":"31","doi-asserted-by":"crossref","unstructured":"31. Kokuzawa A, Nakamura J, Kamata Y, Sato K. Potential role of type I interferon\/IP-10 axis in the pathogenesis of anti-MDA5 antibody-positive dermatomyositis. Clin Exp Rheumatol 41: 275-284, 2023.","DOI":"10.55563\/clinexprheumatol\/em67zx"},{"key":"32","doi-asserted-by":"crossref","unstructured":"32. Fujisawa T, Hozumi H, Yasui H, et al. Clinical significance of serum chitotriosidase level in anti-MDA5 antibody-positive dermatomyositis-associated interstitial lung disease. J Rheumatol 46: 935-942, 2019.","DOI":"10.3899\/jrheum.180825"},{"key":"33","doi-asserted-by":"crossref","unstructured":"33. Enomoto Y, Suzuki Y, Hozumi H, et al. Clinical significance of soluble CD163 in polymyositis-related or dermatomyositis-related interstitial lung disease. Arthritis Res Ther 19: 9, 2017.","DOI":"10.1186\/s13075-016-1214-8"},{"key":"34","doi-asserted-by":"crossref","unstructured":"34. Liang L, Zhang YM, Shen YW, et al. Aberrantly expressed galectin-9 is involved in the immunopathogenesis of anti-MDA5-positive dermatomyositis-associated interstitial lung disease. Front Cell Dev Biol 9: 628128, 2021.","DOI":"10.3389\/fcell.2021.628128"},{"key":"35","doi-asserted-by":"crossref","unstructured":"35. Horai Y, Koga T, Fujikawa K, et al. Serum interferon-\u03b1 is a useful biomarker in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis. Mod Rheumatol 25: 85-89, 2015.","DOI":"10.3109\/14397595.2014.900843"},{"key":"36","doi-asserted-by":"crossref","unstructured":"36. Shi Y, You H, Liu C, et al. Elevated serum B-cell activator factor levels predict rapid progressive interstitial lung disease in anti-melanoma differentiation associated protein 5 antibody positive dermatomyositis. Orphanet J Rare Dis 19: 170, 2024.","DOI":"10.1186\/s13023-024-03153-6"},{"key":"37","doi-asserted-by":"crossref","unstructured":"37. Wang Y, Zhu L, Ju B, et al. Alterations of peripheral blood B cell subsets in Chinese patients with adult idiopathic inflammatory myopathies. Clin Exp Rheumatol 40: 260-266, 2022.","DOI":"10.55563\/clinexprheumatol\/ohsmuj"},{"key":"38","doi-asserted-by":"crossref","unstructured":"38. He J, Liu Z, Cao Y, et al. Single-cell landscape of peripheral immune response in patients with anti-melanoma differentiation-associated gene 5 dermatomyositis. Rheumatology (Oxford) 63: 2284-2294, 2024.","DOI":"10.1093\/rheumatology\/kead597"},{"key":"39","doi-asserted-by":"crossref","unstructured":"39. Ye Y, Chen Z, Jiang S, et al. Single-cell profiling reveals distinct adaptive immune hallmarks in MDA5+ dermatomyositis with therapeutic implications. Nat Commun 13: 6458, 2022.","DOI":"10.1038\/s41467-022-34145-4"},{"key":"40","doi-asserted-by":"crossref","unstructured":"40. Espinosa-Ortega F, Holmqvist M, Alexanderson H, et al. Comparison of autoantibody specificities tested by a line blot assay and immunoprecipitation-based algorithm in patients with idiopathic inflammatory myopathies. Ann Rheum Dis 78: 858-860, 2019.","DOI":"10.1136\/annrheumdis-2018-214690"},{"key":"41","doi-asserted-by":"crossref","unstructured":"41. Mo Y, Ye Y, Peng L, Sun X, Zhong X, Wu R. The central helicase domain holds the major conformational epitopes of melanoma differentiation-associated gene 5 autoantibodies. Rheumatology (Oxford) 63: 1456-1465, 2024.","DOI":"10.1093\/rheumatology\/kead397"},{"key":"42","doi-asserted-by":"crossref","unstructured":"42. Van Gompel E, Demirdal D, Fernandes-Cerqueira C, et al. Autoantibodies against the melanoma differentiation-associated protein 5 in patients with dermatomyositis target the helicase domains. Rheumatology (Oxford) 63: 1466-1473, 2024.","DOI":"10.1093\/rheumatology\/kead400"},{"key":"43","doi-asserted-by":"crossref","unstructured":"43. Yamaguchi K, Poland P, Zhu L, et al. Comparative B cell epitope profiling in Japanese and North American cohorts of MDA5<sup>+<\/sup> dermatomyositis patients reveals a direct association between immune repertoire and pulmonary mortality. Rheumatology (Oxford) 64: 2953-2960, 2025.","DOI":"10.1093\/rheumatology\/keae466"},{"key":"44","doi-asserted-by":"crossref","unstructured":"44. Kang DC, Gopalkrishnan RV, Wu Q, Jankowsky E, Pyle AM, Fisher PB. <i>mda-5<\/i>: An interferon-inducible putative RNA helicase with double-stranded RNA-dependent ATPase activity and melanoma growth-suppressive properties. Proc Natl Acad Sci U S A 99: 637-642, 2002.","DOI":"10.1073\/pnas.022637199"},{"key":"45","doi-asserted-by":"crossref","unstructured":"45. Coutant F, Bachet R, Pin JJ, Alonzo M, Miossec P. Monoclonal antibodies from B cells of patients with anti-MDA5 antibody-positive dermatomyositis directly stimulate interferon gamma production. J Autoimmun 130: 102831, 2022.","DOI":"10.1016\/j.jaut.2022.102831"},{"key":"46","doi-asserted-by":"crossref","unstructured":"46. Tsuji H, Nakashima R, Hosono Y, et al. 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. Arthritis Rheumatol 72: 488-498, 2020.","DOI":"10.1002\/art.41105"},{"key":"47","doi-asserted-by":"crossref","unstructured":"47. Lv C, You H, Xu L, et al. Coexistence of anti-Ro52 antibodies in anti-MDA5 antibody-positive dermatomyositis is highly associated with rapidly progressive interstitial lung disease and mortality risk. J Rheumatol 50: 219-226, 2023.","DOI":"10.3899\/jrheum.220139"},{"key":"48","doi-asserted-by":"crossref","unstructured":"48. Arase N, Tsuji H, Takamatsu H, et al. Cell surface-expressed Ro52\/IgG\/HLA-DR complex is targeted by autoantibodies in patients with inflammatory myopathies. J Autoimmun 126: 102774, 2022.","DOI":"10.1016\/j.jaut.2021.102774"},{"key":"49","doi-asserted-by":"crossref","unstructured":"49. Wang Z, Zheng Q, Xuan W, et al. Short-term effectiveness of baricitinib in children with refractory and\/or severe juvenile dermatomyositis. Front Pediatr 10: 962585, 2022.","DOI":"10.3389\/fped.2022.962585"},{"key":"50","doi-asserted-by":"crossref","unstructured":"50. Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R. Antimelanoma differentiation-associated gene 5 antibody: expanding the clinical spectrum in North American patients with dermatomyositis. J Rheumatol 44: 319-325, 2017.","DOI":"10.3899\/jrheum.160682"},{"key":"51","doi-asserted-by":"crossref","unstructured":"51. Tsuji H, Aitani Y, Koshida Y, et al. Differences in organ damage based on age at onset in idiopathic inflammatory myopathies: a retrospective multicenter MYKO study. Intern Med 64: 3214-3225, 2025.","DOI":"10.2169\/internalmedicine.5447-25"},{"key":"52","doi-asserted-by":"crossref","unstructured":"52. Tanizawa K, Handa T, Nakashima R, et al. HRCT features of interstitial lung disease in dermatomyositis with anti-CADM-140 antibody. Respir Med 105: 1380-1387, 2011.","DOI":"10.1016\/j.rmed.2011.05.006"},{"key":"53","doi-asserted-by":"crossref","unstructured":"53. Kobayashi N, Takezaki S, Kobayashi I, et al. Clinical and laboratory features of fatal rapidly progressive interstitial lung disease associated with juvenile dermatomyositis. Rheumatology (Oxford) 54: 784-791, 2015.","DOI":"10.1093\/rheumatology\/keu385"},{"key":"54","doi-asserted-by":"crossref","unstructured":"54. Iwata N, Nakaseko H, Kohagura T, et al. Clinical subsets of juvenile dermatomyositis classified by myositis-specific autoantibodies: Experience at a single center in Japan. Mod Rheumatol 29: 802-807, 2019.","DOI":"10.1080\/14397595.2018.1511025"},{"key":"55","doi-asserted-by":"crossref","unstructured":"55. Ueki M, Kobayashi I, Takezaki S, et al. Myositis-specific autoantibodies in Japanese patients with juvenile idiopathic inflammatory myopathies. Mod Rheumatol 29: 351-356, 2019.","DOI":"10.1080\/14397595.2018.1452353"},{"key":"56","doi-asserted-by":"crossref","unstructured":"56. Tansley SL, Simou S, Shaddick G, et al. Autoantibodies in juvenile-onset myositis: their diagnostic value and associated clinical phenotype in a large UK cohort. J Autoimmun 84: 55-64, 2017.","DOI":"10.1016\/j.jaut.2017.06.007"},{"key":"57","doi-asserted-by":"crossref","unstructured":"57. Deakin CT, Yasin SA, Simou S, et al.; the UK Juvenile Dermatomyositis Research Group. Muscle biopsy findings in combination with myositis-specific autoantibodies aid prediction of outcomes in juvenile dermatomyositis. Arthritis Rheumatol 68: 2806-2816, 2016.","DOI":"10.1002\/art.39753"},{"key":"58","doi-asserted-by":"crossref","unstructured":"58. Yamasaki Y, Kobayashi N, Akioka S, et al. Clinical impact of myositis-specific autoantibodies on long-term prognosis of juvenile idiopathic inflammatory myopathies: multicentre study. Rheumatology (Oxford) 60: 4821-4831, 2021.","DOI":"10.1093\/rheumatology\/keab108"},{"key":"59","unstructured":"59. Yokota S. The Ministry of Health, Labour and Welfare of Japan Study Group Report on Severe Pediatric Rheumatic Disease. Ministry of Health, Labour and Welfare, Tokyo [Internet]. 2010 [cited 2025 Nov 15]. [14-18]. Available from: https:\/\/mhlw-grants.niph.go.jp\/system\/files\/2010\/103061\/201023008B\/201023008B0001.pdf (in Japanese)."},{"key":"60","doi-asserted-by":"crossref","unstructured":"60. Kobayashi I, Akioka S, Kobayashi N, et al. Clinical practice guidance for juvenile dermatomyositis (JDM) 2018-Update. Mod Rheumatol 30: 411-423, 2020.","DOI":"10.1080\/14397595.2020.1718866"},{"key":"61","doi-asserted-by":"crossref","unstructured":"61. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292: 344-347, 1975.","DOI":"10.1056\/NEJM197502132920706"},{"key":"62","doi-asserted-by":"crossref","unstructured":"62. Sontheimer RD. Would a new name hasten the acceptance of amyopathic dermatomyositis (dermatomyositis sin\u00e9 myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 46: 626-636, 2002.","DOI":"10.1067\/mjd.2002.120621"},{"key":"63","doi-asserted-by":"crossref","unstructured":"63. Lundberg IE, Tj\u00e4rnlund A, Bottai M, et al.; The International Myositis Classification Critera Project consortium, The Euromyositis register and The Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland). 2017 European League Against Rheumatism\/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis 76: 1955-1964, 2017.","DOI":"10.1136\/annrheumdis-2017-212786"},{"key":"64","doi-asserted-by":"crossref","unstructured":"64. Patel B, Khan N, Werth VP. Applicability of EULAR\/ACR classification criteria for dermatomyositis to amyopathic disease. J Am Acad Dermatol 79: 77-83.e71, 2018.","DOI":"10.1016\/j.jaad.2017.12.055"},{"key":"65","doi-asserted-by":"crossref","unstructured":"65. Kobayashi I, Ono S, Kawamura N, et al. KL-6 is a potential marker for interstitial lung disease associated with juvenile dermatomyositis. J Pediatr 138: 274-276, 2001.","DOI":"10.1067\/mpd.2001.110324"},{"key":"66","doi-asserted-by":"crossref","unstructured":"66. Matsushita T, Mizumaki K, Kano M, et al. Antimelanoma differentiation-associated protein 5 antibody level is a novel tool for monitoring disease activity in rapidly progressive interstitial lung disease with dermatomyositis. Br J Dermatol 176: 395-402, 2017.","DOI":"10.1111\/bjd.14882"},{"key":"67","doi-asserted-by":"crossref","unstructured":"67. So J, So H, Wong VT, et al. Predictors of rapidly progressive interstitial lung disease and mortality in patients with autoantibodies against melanoma differentiation-associated protein 5 dermatomyositis. Rheumatology (Oxford) 61: 4437-4444, 2022.","DOI":"10.1093\/rheumatology\/keac094"},{"key":"68","doi-asserted-by":"crossref","unstructured":"68. Xu L, You H, Wang L, et al. Identification of three different phenotypes in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis patients: implications for prediction of rapidly progressive interstitial lung disease. Arthritis Rheumatol 75: 609-619, 2023.","DOI":"10.1002\/art.42308"},{"key":"69","doi-asserted-by":"crossref","unstructured":"69. Gono T, Masui K, Nishina N, et al.; the Multicenter Retrospective Cohort of Japanese Patients with Myositis-Associated ILD (JAMI) Investigators. Risk prediction modeling based on a combination of initial serum biomarker levels in polymyositis\/dermatomyositis-associated interstitial lung disease. Arthritis Rheumatol 73: 677-686, 2021.","DOI":"10.1002\/art.41566"},{"key":"70","doi-asserted-by":"crossref","unstructured":"70. Koga T, Fujikawa K, Horai Y, et al. The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM. Rheumatology (Oxford) 51: 1278-1284, 2012.","DOI":"10.1093\/rheumatology\/ker518"},{"key":"71","unstructured":"71. Kameda H, Nagasawa H, Ogawa H, et al. Combination therapy with corticosteroids, cyclosporin A, and intravenous pulse cyclophosphamide for acute\/subacute interstitial pneumonia in patients with dermatomyositis. J Rheumatol 32: 1719-1726, 2005."},{"key":"72","doi-asserted-by":"crossref","unstructured":"72. Sasai T, Nakashima R, Tsuji H, et al. Long-term prognosis of antimelanoma differentiation-associated gene 5-positive dermatomyositis with interstitial lung disease. J Rheumatol 50: 1454-1461, 2023.","DOI":"10.3899\/jrheum.2023-0371"},{"key":"73","doi-asserted-by":"crossref","unstructured":"73. Kurasawa K, Arai S, Namiki Y, et al. Tofacitinib for refractory interstitial lung diseases in anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis. Rheumatology (Oxford) 57: 2114-2119, 2018.","DOI":"10.1093\/rheumatology\/key188"},{"key":"74","doi-asserted-by":"crossref","unstructured":"74. Li S, Li S, Wang J, et al. Efficacy and safety of tofacitinib in anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis. J Clin Rheumatol 29: 281-284, 2023.","DOI":"10.1097\/RHU.0000000000002010"},{"key":"75","doi-asserted-by":"crossref","unstructured":"75. Silveira MG, Selva-O&apos;Callaghan A, Ramos-Terrades N, Arredondo-Agudelo KV, Labrador-Horrillo M, Bravo-Masgoret C. Anti-MDA5 dermatomyositis and progressive interstitial pneumonia. QJM 109: 49-50, 2016.","DOI":"10.1093\/qjmed\/hcv068"},{"key":"76","doi-asserted-by":"crossref","unstructured":"76. Abe Y, Kusaoi M, Tada K, Yamaji K, Tamura N. Successful treatment of anti-MDA5 antibody-positive refractory interstitial lung disease with plasma exchange therapy. Rheumatology (Oxford) 59: 767-771, 2020.","DOI":"10.1093\/rheumatology\/kez357"},{"key":"77","doi-asserted-by":"publisher","unstructured":"77. Teruya A, Kawamura K, Ichikado K, Sato S, Yasuda Y, Yoshioka M. Successful polymyxin B hemoperfusion treatment associated with serial reduction of serum anti-CADM-140\/MDA5 antibody levels in rapidly progressive interstitial lung disease with amyopathic dermatomyositis. Chest 144: 1934-1936, 2013.","DOI":"10.1378\/chest.13-0186"},{"key":"78","doi-asserted-by":"crossref","unstructured":"78. Ichiyasu H, Horio Y, Masunaga A, et al. Efficacy of direct hemoperfusion using polymyxin B-immobilized fiber column (PMX-DHP) in rapidly progressive interstitial pneumonias: results of a historical control study and a review of previous studies. Ther Adv Respir Dis 11: 261-275, 2017.","DOI":"10.1177\/1753465817708950"},{"key":"79","doi-asserted-by":"crossref","unstructured":"79. Rovin BH, Furie R, Latinis K, et al.; LUNAR Investigator Group. Efficacy and safety of rituximab in patients with active proliferative lupus nephritis: the Lupus Nephritis Assessment with Rituximab study. Arthritis Rheum 64: 1215-1226, 2012.","DOI":"10.1002\/art.34359"},{"key":"80","doi-asserted-by":"crossref","unstructured":"80. Aksoy S, Harputluoglu H, Kilickap S, et al. Rituximab-related viral infections in lymphoma patients. Leuk Lymphoma 48: 1307-1312, 2007.","DOI":"10.1080\/10428190701411441"},{"key":"81","doi-asserted-by":"crossref","unstructured":"81. Matsushita T, Fujimoto M, Hasegawa M, et al. Inhibitory role of CD19 in the progression of experimental autoimmune encephalomyelitis by regulating cytokine response. Am J Pathol 168: 812-821, 2006.","DOI":"10.2353\/ajpath.2006.050923"},{"key":"82","unstructured":"82. Markatseli TE, Kaltsonoudis ES, Voulgari PV, Zioga A, Drosos AA. Induction of psoriatic skin lesions in a patient with rheumatoid arthritis treated with rituximab. Clin Exp Rheumatol 27: 996-998, 2009."},{"key":"83","doi-asserted-by":"crossref","unstructured":"83. So H, Wong VTL, Lao VWN, Pang HT, Yip RML. Rituximab for refractory rapidly progressive interstitial lung disease related to anti-MDA5 antibody-positive amyopathic dermatomyositis. Clin Rheumatol 37: 1983-1989, 2018.","DOI":"10.1007\/s10067-018-4122-2"},{"key":"84","doi-asserted-by":"crossref","unstructured":"84. Hata K, Kotani T, Matsuda S, et al. Aggressive multi-combination therapy for anti-MDA5 antibody-positive dermatomyositis-rapidly progressive interstitial lung disease. Int J Rheum Dis 27: e14999, 2024.","DOI":"10.1111\/1756-185X.14999"},{"key":"85","doi-asserted-by":"crossref","unstructured":"85. Manghani M, Lim W, Chai GT, Lim XR, Chua CG. Combination therapy with methylprednisolone, rituximab, and tofacitinib in antimelanoma differentiation-associated 5 gene dermatomyositis with rapidly progressive interstitial lung disease. Rheumatol Autoimmun 4: 122-125, 2024.","DOI":"10.1002\/rai2.12112"},{"key":"86","doi-asserted-by":"crossref","unstructured":"86. Mao MM, Xia S, Guo BP, et al. Ultra-low dose rituximab as add-on therapy in anti-MDA5-positive patients with polymyositis \/dermatomyositis associated ILD. Respir Med 172: 105983, 2020.","DOI":"10.1016\/j.rmed.2020.105983"},{"key":"87","doi-asserted-by":"crossref","unstructured":"87. Wang LM, Yang QH, Zhang L, et al. Intravenous immunoglobulin for interstitial lung diseases of anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Rheumatology (Oxford) 61: 3704-3710, 2022.","DOI":"10.1093\/rheumatology\/keab928"},{"key":"88","doi-asserted-by":"crossref","unstructured":"88. Holzer MT, Nies JF, Oqueka T, Huber TB, K\u00f6tter I, Krusche M. Successful rescue therapy with daratumumab in rapidly progressive interstitial lung disease caused by MDA5-positive dermatomyositis. Chest 163: e1-e5, 2023.","DOI":"10.1016\/j.chest.2022.08.2209"},{"key":"89","doi-asserted-by":"publisher","unstructured":"89. Ostendorf L, Muench F, Thorm\u00e4hlen L, et al. Rescue combination treatment of anti-MDA5-associated ARDS with daratumumab. RMD Open 9: e003238, 2023.","DOI":"10.1136\/rmdopen-2023-003238"},{"key":"90","doi-asserted-by":"crossref","unstructured":"90. Hagen M, Bucci L, B\u00f6ltz S, et al. BCMA-targeted T-cell-engager therapy for autoimmune disease. N Engl J Med 391: 867-869, 2024.","DOI":"10.1056\/NEJMc2408786"},{"key":"91","doi-asserted-by":"publisher","unstructured":"91. M\u00fcller F, Boeltz S, Knitza J, et al. CD19-targeted CAR T cells in refractory antisynthetase syndrome. Lancet 401: 815-818, 2023.","DOI":"10.1016\/S0140-6736(23)00023-5"},{"key":"92","doi-asserted-by":"crossref","unstructured":"92. Soares RB, Gabr JB, Ash M, Hosler G. Anifrolumab in refractory dermatomyositis and antisynthetase syndrome. Case Rep Rheumatol 2025: 5560523, 2025.","DOI":"10.1155\/crrh\/5560523"},{"key":"93","doi-asserted-by":"crossref","unstructured":"93. Ang PS, Ezenwa E, Ko K, Hoffman MD. Refractory dermatomyositis responsive to anifrolumab. JAAD Case Rep 43: 27-29, 2024.","DOI":"10.1016\/j.jdcr.2023.10.023"},{"key":"94","doi-asserted-by":"crossref","unstructured":"94. Kobayashi I, Yamada M, Takahashi Y, et al. Interstitial lung disease associated with juvenile dermatomyositis: clinical features and efficacy of cyclosporin A. Rheumatology (Oxford) 42: 371-374, 2003.","DOI":"10.1093\/rheumatology\/keg040"},{"key":"95","doi-asserted-by":"crossref","unstructured":"95. Sabbagh S, Almeida de Jesus A, Hwang S, et al. Treatment of anti-MDA5 autoantibody-positive juvenile dermatomyositis using tofacitinib. Brain 142: e59, 2019.","DOI":"10.1093\/brain\/awz293"},{"key":"96","doi-asserted-by":"crossref","unstructured":"96. Chan Ng PLP, Mopur A, Goh DYT, et al. Janus kinase inhibition in induction treatment of anti-MDA5 juvenile dermatomyositis-associated rapidly progressive interstitial lung disease. Int J Rheum Dis 25: 228-231, 2022.","DOI":"10.1111\/1756-185X.14258"},{"key":"97","doi-asserted-by":"crossref","unstructured":"97. Xue Y, Zhang J, Deng J, et al. Efficiency of tofacitinib in refractory interstitial lung disease among anti-MDA5 positive juvenile dermatomyositis patients. Ann Rheum Dis 82: 1499-1501, 2023.","DOI":"10.1136\/ard-2023-223919"},{"key":"98","doi-asserted-by":"crossref","unstructured":"98. Yamazaki S, Shimizu M, Yakabe A, et al. Successful treatment with tofacitinib for anti-melanoma differentiation-associated gene 5 antibody-positive juvenile dermatomyositis: case reports and review of the literature. Immunol Med 47: 110-117, 2024.","DOI":"10.1080\/25785826.2024.2336687"},{"key":"99","doi-asserted-by":"crossref","unstructured":"99. Nishi K, Ogura M, Tamai N, et al. Successful rituximab treatment for severe rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive juvenile dermatomyositis: a case report and literature review. Pediatr Rheumatol Online J 20: 60, 2022.","DOI":"10.1186\/s12969-022-00723-5"},{"key":"100","doi-asserted-by":"crossref","unstructured":"100. Peskin M, Mostowy M, Velez J, Perron M, Kurian J, Wahezi DM. Clinical improvement in early onset interstitial lung disease using rituximab in children with antimelanoma differentiation-associated gene 5-positive juvenile dermatomyositis. 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