{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T04:18:48Z","timestamp":1793852328441,"version":"4.6.1"},"reference-count":43,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,1,27]],"date-time":"2020-01-27T00:00:00Z","timestamp":1580083200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["16H05341"],"award-info":[{"award-number":["16H05341"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP25293222"],"award-info":[{"award-number":["JP25293222"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>\n                      Interstitial lung disease (\n                      <jats:styled-content style=\"fixed-case\">ILD<\/jats:styled-content>\n                      ) accompanied by anti\u2013melanoma differentiation\u2013associated gene 5 (anti\u2013\n                      <jats:styled-content style=\"fixed-case\">MDA<\/jats:styled-content>\n                      \u20105)\u2013positive dermatomyositis (\n                      <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>\n                      ) is often rapidly progressive and associated with poor prognosis. Because there is no established treatment, we undertook this study to prospectively evaluate the efficacy and safety of a combined immunosuppressive regimen for anti\u2013\n                      <jats:styled-content style=\"fixed-case\">MDA<\/jats:styled-content>\n                      \u20105\u2013positive\n                      <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>\n                      patients with\n                      <jats:styled-content style=\"fixed-case\">ILD<\/jats:styled-content>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Adult Japanese patients with new\u2010onset anti\u2013\n                      <jats:styled-content style=\"fixed-case\">MDA<\/jats:styled-content>\n                      \u20105\u2013positive\n                      <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>\n                      with\n                      <jats:styled-content style=\"fixed-case\">ILD<\/jats:styled-content>\n                      (n = 29) were enrolled at multiple study centers from 2014 to 2017. They were treated with a regimen of high\u2010dose glucocorticoids (\n                      <jats:styled-content style=\"fixed-case\">GC<\/jats:styled-content>\n                      s), tacrolimus, and intravenous cyclophosphamide (\n                      <jats:styled-content style=\"fixed-case\">IV CYC<\/jats:styled-content>\n                      ). Plasmapheresis was used if a patient's condition worsened after the regimen started. The primary end point was 6\u2010month survival, which was compared between this group of patients and a historical control group (n = 15) consisting of anti\u2013\n                      <jats:styled-content style=\"fixed-case\">MDA<\/jats:styled-content>\n                      \u20105\u2013positive\n                      <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>\n                      patients with\n                      <jats:styled-content style=\"fixed-case\">ILD<\/jats:styled-content>\n                      who received step\u2010up treatment (high\u2010dose\n                      <jats:styled-content style=\"fixed-case\">GC<\/jats:styled-content>\n                      and stepwise addition of immunosuppressant). Secondary end points were 12\u2010month survival rate, adverse events, and changes in laboratory data.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The combined immunosuppressive regimen group showed significantly higher 6\u2010month survival rates than the step\u2010up treatment group (89% versus 33%;\n                      <jats:italic>P<\/jats:italic>\n                      &lt; 0.0001). Over a period of 52 weeks, improvements in anti\u2013\n                      <jats:styled-content style=\"fixed-case\">MDA<\/jats:styled-content>\n                      \u20105 titers, serum ferritin levels, vital capacity, and chest high\u2010resolution computed tomography scores were observed. The combined immunosuppressive regimen group received\n                      <jats:styled-content style=\"fixed-case\">IV CYC<\/jats:styled-content>\n                      nearly 20 days earlier with shorter intervals and tended to receive plasmapheresis more often than patients undergoing step\u2010up treatment. Cytomegalovirus reactivation was frequently observed over 52 weeks.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      A combined immunosuppressive regimen is effective for anti\u2013\n                      <jats:styled-content style=\"fixed-case\">MDA<\/jats:styled-content>\n                      \u20105\u2013positive\n                      <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>\n                      patients with\n                      <jats:styled-content style=\"fixed-case\">ILD<\/jats:styled-content>\n                      . Plasmapheresis can be used for additional effect in intractable disease. Patients should be carefully monitored for opportunistic infections during treatment.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.41105","type":"journal-article","created":{"date-parts":[[2019,9,16]],"date-time":"2019-09-16T05:43:08Z","timestamp":1568612588000},"page":"488-498","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":330,"title":["Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High\u2010Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti\u2013Melanoma Differentiation\u2013Associated Gene 5\u2013Positive Dermatomyositis"],"prefix":"10.1002","volume":"72","author":[{"given":"Hideaki","family":"Tsuji","sequence":"first","affiliation":[{"name":"Kyoto University Graduate School of Medicine  Kyoto 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