{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T15:08:57Z","timestamp":1761491337117,"version":"build-2065373602"},"reference-count":19,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2021,12,13]],"date-time":"2021-12-13T00:00:00Z","timestamp":1639353600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100010101","name":"Taichung Veterans General Hospital","doi-asserted-by":"publisher","award":["TCVGH-YMCT1109114"],"award-info":[{"award-number":["TCVGH-YMCT1109114"]}],"id":[{"id":"10.13039\/501100010101","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Anti-melanoma differentiation-associated protein 5 (MDA5)-positive rapidly progressive interstitial lung disease (RP-ILD) is associated with poor prognosis, and the most effective therapeutic intervention has not been established. Herein we report a case of a 45-year-old female patient who presented with myalgia, Gottron\u2019s papules with ulceration, and dyspnea on exertion which became aggravated within weeks. Laboratory examination and electromyography confirmed myopathy changes, and a survey of myositis-specific antibodies was strongly positive for anti-MDA5 antibody. High-resolution chest tomography suggested organizing pneumonia with rapidly progressive changes within the first month after diagnosis of the disease. Anti-MDA5-associated dermatomyositis with RP-ILD was diagnosed. Following combination therapy with rituximab, tofacitinib and pirfenidone, clinical symptoms, including cutaneous manifestation, respiratory conditions and radiographic changes, showed significant and sustainable improvement. To our knowledge, this is the first reported case of anti-MDA5-associated dermatomyositis with RP-ILD successfully treated with the combination of rituximab, tofacitinib, and pirfenidone.<\/jats:p>","DOI":"10.3390\/medicina57121358","type":"journal-article","created":{"date-parts":[[2021,12,14]],"date-time":"2021-12-14T01:20:41Z","timestamp":1639444841000},"page":"1358","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Combination Therapy with Rituximab, Tofacitinib and Pirfenidone in a Patient with Rapid Progressive Interstitial Lung Disease (RP-ILD) Due to MDA5 Antibody-Associated Dermatomyositis: A Case Report"],"prefix":"10.3390","volume":"57","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9520-2665","authenticated-orcid":false,"given":"Tsai-Hung","family":"Yen","sequence":"first","affiliation":[{"name":"Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung 40705, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5948-7306","authenticated-orcid":false,"given":"Chih-Wei","family":"Tseng","sequence":"additional","affiliation":[{"name":"Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung 40705, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kao-Lun","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Radiology, Taichung Veterans General Hospital, Taichung 40705, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9416-4094","authenticated-orcid":false,"given":"Pin-Kuei","family":"Fu","sequence":"additional","affiliation":[{"name":"Department of Critical Care Medicine, Taichung Veterans General Hospital, Taichung 40705, Taiwan"},{"name":"Integrated Care Center of Interstitial Lung Disease, Taichung Veterans General Hospital, Taichung 40705, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,12,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/j.rmed.2016.10.019","article-title":"Comprehensive assessment of myositis-specific autoantibodies in polymyositis\/dermatomyositis-associated interstitial lung disease","volume":"121","author":"Hozumi","year":"2016","journal-title":"Respir. 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