{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T12:33:42Z","timestamp":1781699622811,"version":"3.54.5"},"reference-count":28,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2020,11,27]],"date-time":"2020-11-27T00:00:00Z","timestamp":1606435200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p><jats:bold>Introduction:<\/jats:bold> Patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive amyopathic dermatomyositis (ADM) often develop rapidly progressive interstitial lung diseases (RP-ILD), with poor treatment success. Many studies have shown that this is the main cause of death in patients with anti-MDA5 antibody-positive ADM.<\/jats:p><jats:p><jats:bold>Case Presentation:<\/jats:bold> A 37-years-old woman developed a cough, shortness of breath, and a rash on both hands, which resembled Gottron's signs. Upon laboratory examination, the results were as follows: antinuclear antibody (ANA) positive; anti-Ro52 antibody positive; and anti-MDA5 antibody positive. Pulmonary high-resolution CT (HRCT) scan showed pulmonary interstitial inflammatory changes, and mediastinal and subcutaneous emphysema. She was finally diagnosed with anti-MDA5 antibody-positive ADM accompanied by RP-ILD. She was first given high-dose-steroid pulse therapy with methylprednisolone (500 mg per day for 3 days) followed by methylprednisolone (40 mg, daily), cyclosporine A (100 mg, twice per day), and hydroxychloroquine (200 mg, twice per day). Since her discharge from our hospital in March of 2018, she has maintained the methylprednisolone therapy (tapered to 10 mg daily), cyclosporine A (100 mg, twice per day), and hydroxychloroquine (200 mg, twice per day).<\/jats:p><jats:p><jats:bold>Outcomes:<\/jats:bold> Pulmonary HRCT scans taken on 4, 9, and 26 months after her discharge from our hospital showed that the interstitial pneumonitis had significantly improved and that mediastinal and subcutaneous emphysema had been gradually absorbed. The patient can now participate in regular work and activities of daily living.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> The treatment of methylprednisolone pulse therapy combined with cyclosporine A and hydroxychloroquine may be an option for the RP-ILD accompanied by anti-MDA-positive ADM. After the acute phase, this combination therapy strategy is helpful to the disease control of patients.<\/jats:p>","DOI":"10.3389\/fmed.2020.610554","type":"journal-article","created":{"date-parts":[[2020,11,27]],"date-time":"2020-11-27T07:02:03Z","timestamp":1606460523000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":3,"title":["Case Report: Treatment of Anti-MDA5-Positive Amyopathic Dermatomyositis Accompanied by a Rapidly Progressive Interstitial Lung Diseases With Methylprednisolone Pulse Therapy Combined With Cyclosporine A and Hydroxychloroquine"],"prefix":"10.3389","volume":"7","author":[{"given":"Qia-Chun","family":"Zhang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min-Ying","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhi-Xin","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yimin Talia","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chang-Song","family":"Lin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiang","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2020,11,27]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"2607","DOI":"10.1002\/art.21291","article-title":"International consensus guidelines for trials of therapies in the idiopathic inflammatory myopathies","volume":"52","author":"Oddis","year":"2005","journal-title":"Arthritis Rheum."},{"key":"B2","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1038\/nrrheum.2018.41","article-title":"Classification of myositis","volume":"14","author":"Lundberg","year":"2018","journal-title":"Nat Rev Rheumatol."},{"key":"B3","doi-asserted-by":"publisher","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":"B4","doi-asserted-by":"publisher","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":"B5","doi-asserted-by":"publisher","first-page":"231","DOI":"10.1097\/01.md.0000173991.74008.b0","article-title":"Novel classification of idiopathic inflammatory myopathies based on overlap syndrome features and autoantibodies: analysis of 100 French Canadian patients","volume":"84","author":"Troyanov","year":"2005","journal-title":"Medicine (Baltimore)."},{"key":"B6","doi-asserted-by":"publisher","first-page":"959","DOI":"10.1016\/0190-9622(91)70153-S","article-title":"Amyopathic dermatomyositis (dermatomyositis sin\u00e9 myositis). Presentation of six new cases and review of the literature","volume":"24","author":"Euwer","year":"1991","journal-title":"J Am Acad Dermatol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"626","DOI":"10.1067\/mjd.2002.120621","article-title":"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?","volume":"46","author":"Sontheimer","year":"2002","journal-title":"J Am Acad Dermatol."},{"key":"B8","doi-asserted-by":"publisher","first-page":"1591","DOI":"10.1136\/annrheumdis-2014-205278","article-title":"Basiliximab may improve the survival rate of rapidly progressive interstitial pneumonia in patients with clinically amyopathic dermatomyositis with anti-MDA5 antibody","volume":"73","author":"Zou","year":"2014","journal-title":"Ann Rheum Dis."},{"key":"B9","doi-asserted-by":"publisher","first-page":"1005","DOI":"10.1183\/09031936.06.00038806","article-title":"Interstitial lung diseases associated with amyopathic dermatomyositis","volume":"28","author":"Suda","year":"2006","journal-title":"Eur Respir J."},{"key":"B10","doi-asserted-by":"publisher","first-page":"646","DOI":"10.1164\/ajrccm.161.2.ats3-00","article-title":"Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS)","volume":"161","year":"2000","journal-title":"Am J Respir Crit Care Med."},{"key":"B11","doi-asserted-by":"publisher","first-page":"1278","DOI":"10.1093\/rheumatology\/ker518","article-title":"The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM","volume":"51","author":"Koga","year":"2012","journal-title":"Rheumatology (Oxford)."},{"key":"B12","doi-asserted-by":"publisher","first-page":"1563","DOI":"10.1093\/rheumatology\/kes102","article-title":"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","volume":"51","author":"Gono","year":"2012","journal-title":"Rheumatology (Oxford)."},{"key":"B13","doi-asserted-by":"publisher","first-page":"1971","DOI":"10.1007\/s00296-019-04398-2","article-title":"Clinical spectrum and therapeutics in Canadian patients with anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis: a case-based review","volume":"39","author":"Huang","year":"2019","journal-title":"Rheumatol Int."},{"key":"B14","doi-asserted-by":"publisher","first-page":"190","DOI":"10.3109\/s10165-012-0637-6","article-title":"Early diagnosis and treatment for remission of clinically amyopathic dermatomyositis complicated by rapid progress interstitial lung disease: a report of two cases","volume":"23","author":"Horai","year":"2013","journal-title":"Mod Rheumatol."},{"key":"B15","doi-asserted-by":"publisher","first-page":"1293","DOI":"10.1007\/s00296-018-3991-7","article-title":"Rapidly progressive interstitial lung disease due to anti-MDA5 antibodies without skin involvement: a case report and literature review","volume":"38","author":"Gonz\u00e1lez-Moreno","year":"2018","journal-title":"Rheumatol Int."},{"key":"B16","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1186\/s12890-018-0622-8","article-title":"MDA-5 associated rapidly progressive interstitial lung disease with recurrent Pneumothoraces: a case report","volume":"18","author":"Alqatari","year":"2018","journal-title":"BMC Pulm Med."},{"key":"B17","doi-asserted-by":"publisher","first-page":"9","DOI":"10.4137\/CCRPM.S23313","article-title":"Recent treatment of interstitial lung disease with idiopathic inflammatory myopathies","volume":"9","author":"Kawasumi","year":"2015","journal-title":"Clin Med Insights Circ Respir Pulm Med."},{"key":"B18","first-page":"803","article-title":"Clinical features of thirty-two patients with anti-melanoma differentiation-associated gene 5 antibodies","volume":"37","author":"Huang","year":"2019","journal-title":"Clin Exp Rheumatol."},{"key":"B19","doi-asserted-by":"publisher","first-page":"2193","DOI":"10.1002\/art.24621","article-title":"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","volume":"60","author":"Sato","year":"2009","journal-title":"Arthritis Rheum."},{"key":"B20","doi-asserted-by":"publisher","first-page":"1571","DOI":"10.1002\/art.21023","article-title":"Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis","volume":"52","author":"Sato","year":"2005","journal-title":"Arthritis Rheum."},{"key":"B21","doi-asserted-by":"publisher","first-page":"1316","DOI":"10.1002\/acr.21985","article-title":"Utility of anti-melanoma differentiation-associated gene 5 antibody measurement in identifying patients with dermatomyositis and a high risk for developing rapidly progressive interstitial lung disease: a review of the literature and a meta-analysis","volume":"65","author":"Chen","year":"2013","journal-title":"Arthritis Care Res (Hoboken)."},{"key":"B22","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1111\/1346-8138.14092","article-title":"Associations between anti-melanoma differentiation-associated gene 5 antibody and demographics, clinical characteristics and laboratory results of patients with dermatomyositis: a systematic meta-analysis","volume":"45","author":"Li","year":"2018","journal-title":"J Dermatol."},{"key":"B23","doi-asserted-by":"publisher","first-page":"625","DOI":"10.3109\/s10165-011-0558-9","article-title":"Amyopathic dermatomyositis developing rapidly progressive interstitial lung disease with elevation of anti-CADM-140\/MDA5 autoantibodies","volume":"22","author":"Sato","year":"2012","journal-title":"Mod Rheumatol."},{"key":"B24","doi-asserted-by":"publisher","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 (Oxford)."},{"key":"B25","doi-asserted-by":"publisher","first-page":"83","DOI":"10.5152\/eurjrheum.2015.0076","article-title":"High-dose intravenous immunoglobulin therapy for rapidly progressive interstitial pneumonitis accompanied by anti-melanoma differentiation-associated gene 5 antibody-positive amyopathic dermatomyositis","volume":"2","author":"Hamada-Ode","year":"2015","journal-title":"Eur J Rheumatol."},{"key":"B26","doi-asserted-by":"publisher","first-page":"767","DOI":"10.1093\/rheumatology\/kez357","article-title":"Successful treatment of anti-MDA5 antibody-positive refractory interstitial lung disease with plasma exchange therapy","volume":"59","author":"Abe","year":"2020","journal-title":"Rheumatology (Oxford)."},{"key":"B27","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1016\/j.berh.2016.04.006","article-title":"Connective tissue disease-related interstitial lung disease","volume":"30","author":"Demoruelle","year":"2016","journal-title":"Best Pract Res Clin Rheumatol."},{"key":"B28","doi-asserted-by":"publisher","first-page":"e0436","DOI":"10.1097\/MD.0000000000010436","article-title":"Successful treatment of plasma exchange for rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis: a case report","volume":"97","author":"Endo","year":"2018","journal-title":"Medicine (Baltimore)."}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2020.610554\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,11,27]],"date-time":"2020-11-27T07:02:07Z","timestamp":1606460527000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2020.610554\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,11,27]]},"references-count":28,"alternative-id":["10.3389\/fmed.2020.610554"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2020.610554","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,11,27]]},"article-number":"610554"}}