{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,30]],"date-time":"2026-08-30T12:00:42Z","timestamp":1788091242811,"version":"build-2803163510"},"reference-count":31,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,10,30]],"date-time":"2024-10-30T00:00:00Z","timestamp":1730246400000},"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. Pharmacol."],"abstract":"<jats:p>Dermatomyositis (DM) positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, mainly when linked with rapidly progressive interstitial lung disease (RP-ILD), is considered a refractory disease. Our report describes a critical case of clinically amyopathic dermatomyositis (CADM) with RP-ILD that tested positive for both anti-MDA5 and anti-Ro-52 antibodies. The patient showed a limited response to a combined therapy regimen of prednisone, iguratimod, and tacrolimus. However, after adjunct therapy with mycophenolate mofetil (MMF), the patient\u2019s condition was controlled, his serum KL-6 levels decreased, and anti-MDA5 antibodies became negative. During the 68-week follow-up, the patient\u2019s condition remained stable, with a satisfactory quality of life. This report also discusses the potential role of inflammatory cytokines in the pathophysiology of CADM and RP-ILD. Further research is required to confirm these results and investigate the application of MMF in maintenance therapy for CADM-associated RP-ILD.<\/jats:p>","DOI":"10.3389\/fphar.2024.1472667","type":"journal-article","created":{"date-parts":[[2024,10,30]],"date-time":"2024-10-30T05:11:18Z","timestamp":1730265078000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":4,"title":["Mycophenolate mofetil after tacrolimus for refractory clinically amyopathic dermatomyositis: a case report"],"prefix":"10.3389","volume":"15","author":[{"given":"Gui-Chen","family":"Ling","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chang","family":"Su","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying-Ao","family":"Guo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xia","family":"Qiu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jia-Wei","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Xiao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Ya","family":"Xiao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuo","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian-Yong","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing-Jing","family":"Xie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2024,10,30]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1016\/s0162-3109(00)00188-0","article-title":"Mycophenolate mofetil and its mechanisms of action","volume":"47","author":"Allison","year":"2000","journal-title":"Immunopharmacology"},{"key":"B2","doi-asserted-by":"publisher","first-page":"999","DOI":"10.1007\/s10067-020-05339-2","article-title":"Hierarchical cluster analysis of cytokine profiles reveals a cutaneous vasculitis-associated subgroup in dermatomyositis","volume":"40","author":"Bai","year":"2021","journal-title":"Clin. Rheumatol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1007\/s12016-023-08963-3","article-title":"Perspectives on mycophenolate mofetil in the management of autoimmunity","volume":"65","author":"Bhat","year":"2023","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"B4","doi-asserted-by":"publisher","first-page":"102831","DOI":"10.1016\/j.jaut.2022.102831","article-title":"Monoclonal antibodies from B cells of patients with anti-MDA5 antibody-positive dermatomyositis directly stimulate interferon gamma production","volume":"130","author":"Coutant","year":"2022","journal-title":"J. Autoimmun."},{"key":"B5","doi-asserted-by":"publisher","first-page":"1718","DOI":"10.1056\/NEJMoa1908681","article-title":"Nintedanib in progressive fibrosing interstitial lung diseases","volume":"381","author":"Flaherty","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"B6","doi-asserted-by":"publisher","first-page":"597","DOI":"10.1016\/j.jaad.2005.10.041","article-title":"A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sin\u00e9 myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies","volume":"54","author":"Gerami","year":"2006","journal-title":"J. Am. Acad. Dermatol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"2196","DOI":"10.1093\/rheumatology\/keu258","article-title":"Cytokine profiles in polymyositis and dermatomyositis complicated by rapidly progressive or chronic interstitial lung disease","volume":"53","author":"Gono","year":"2014","journal-title":"Rheumatol. Oxf."},{"key":"B8","doi-asserted-by":"publisher","first-page":"1354","DOI":"10.1093\/rheumatology\/keq073","article-title":"Increased ferritin predicts development and severity of acute interstitial lung disease as a complication of dermatomyositis","volume":"49","author":"Gono","year":"2010","journal-title":"Rheumatol. Oxf."},{"key":"B9","doi-asserted-by":"publisher","first-page":"1476","DOI":"10.1016\/j.chest.2023.01.031","article-title":"Diagnosis and management of myositis-associated lung disease","volume":"163","author":"Hallowell","year":"2023","journal-title":"Chest"},{"key":"B10","doi-asserted-by":"publisher","first-page":"107596","DOI":"10.1016\/j.intimp.2021.107596","article-title":"Iguratimod reduces B-cell secretion of immunoglobulin to play a protective role in interstitial lung disease","volume":"97","author":"Han","year":"2021","journal-title":"Int. Immunopharmacol."},{"key":"B11","doi-asserted-by":"publisher","first-page":"559","DOI":"10.1002\/jlb.56.5.559","article-title":"Essential involvement of interleukin-8 (IL-8) in acute inflammation","volume":"56","author":"Harada","year":"1994","journal-title":"J. Leukoc. Biol."},{"key":"B12","doi-asserted-by":"publisher","first-page":"e14999","DOI":"10.1111\/1756-185X.14999","article-title":"Aggressive multi-combination therapy for anti-MDA5 antibody-positive dermatomyositis-rapidly progressive interstitial lung disease","volume":"27","author":"Hata","year":"2024","journal-title":"Int. J. Rheum. Dis."},{"key":"B13","doi-asserted-by":"publisher","first-page":"815245","DOI":"10.1155\/2014\/815245","article-title":"IL-6, IL-8, and IL-10 are associated with hyperferritinemia in rapidly progressive interstitial lung disease with polymyositis\/dermatomyositis","volume":"2014","author":"Kawasumi","year":"2014","journal-title":"Biomed. Res. Int."},{"key":"B14","doi-asserted-by":"publisher","first-page":"776","DOI":"10.1016\/j.jaad.2017.12.010","article-title":"Anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis: a concise review with an emphasis on distinctive clinical features","volume":"78","author":"Kurtzman","year":"2018","journal-title":"J. Am. Acad. Dermatol"},{"key":"B15","doi-asserted-by":"publisher","first-page":"575","DOI":"10.1007\/s40265-021-01487-0","article-title":"Nintedanib: a review in fibrotic interstitial lung diseases","volume":"81","author":"Lamb","year":"2021","journal-title":"Drugs"},{"key":"B16","doi-asserted-by":"publisher","first-page":"e23656","DOI":"10.1097\/MD.0000000000023656","article-title":"The prognostic value of IL-8 for the death of severe or critical patients with COVID-19","volume":"100","author":"Li","year":"","journal-title":"Med. Baltim."},{"key":"B17","doi-asserted-by":"publisher","first-page":"3706","DOI":"10.21037\/apm-20-2134","article-title":"Dynamic changes in serum IL-6, IL-8, and IL-10 predict the outcome of ICU patients with severe COVID-19","volume":"10","author":"Li","year":"","journal-title":"Ann. Palliat. Med."},{"key":"B18","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1038\/s41584-023-01054-9","article-title":"Anti-MDA5 antibody-positive dermatomyositis: pathogenesis and clinical progress","volume":"20","author":"Lu","year":"2024","journal-title":"Nat. Rev. Rheumatol."},{"key":"B19","doi-asserted-by":"publisher","first-page":"310","DOI":"10.1093\/rheumatology\/kez301","article-title":"Exploration of pathomechanism using comprehensive analysis of serum cytokines in polymyositis\/dermatomyositis-interstitial lung disease","volume":"59","author":"Matsuda","year":"2020","journal-title":"Rheumatol. Oxf."},{"key":"B20","doi-asserted-by":"publisher","first-page":"151959","DOI":"10.1016\/j.semarthrit.2022.151959","article-title":"Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: a systematic review","volume":"53","author":"McPherson","year":"2022","journal-title":"Semin. Arthritis Rheum."},{"key":"B21","doi-asserted-by":"publisher","first-page":"279","DOI":"10.1159\/000448846","article-title":"Successful treatment of fibrosing organising pneumonia causing respiratory failure with mycophenolic acid","volume":"92","author":"Paul","year":"2016","journal-title":"Respiration"},{"key":"B22","doi-asserted-by":"publisher","first-page":"776","DOI":"10.1016\/j.semarthrit.2020.03.007","article-title":"Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease","volume":"50","author":"Romero-Bueno","year":"2020","journal-title":"Semin. Arthritis Rheum."},{"key":"B23","doi-asserted-by":"publisher","first-page":"196","DOI":"10.1002\/jca.21833","article-title":"Anti-MDA-5 antibody-positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease treated with therapeutic plasma exchange: a case series","volume":"36","author":"Saito","year":"2021","journal-title":"J. Clin. Apher."},{"key":"B24","doi-asserted-by":"publisher","first-page":"291","DOI":"10.55563\/clinexprheumatol\/8kulbf","article-title":"Intensive induction therapy combining tofacitinib, rituximab and plasma exchange in severe anti-melanoma differentiation-associatedprotein-5 antibody-positive dermatomyositis","volume":"41","author":"Shirai","year":"2023","journal-title":"Clin. Exp. Rheumatol."},{"key":"B25","doi-asserted-by":"publisher","first-page":"3284","DOI":"10.1093\/rheumatology\/keaa123","article-title":"Efficacy of plasma exchange in anti-MDA5-positive dermatomyositis with interstitial lung disease under combined immunosuppressive treatment","volume":"59","author":"Shirakashi","year":"2020","journal-title":"Rheumatol. Oxf."},{"key":"B26","doi-asserted-by":"publisher","first-page":"951","DOI":"10.1016\/0190-9622(93)70136-h","article-title":"Cutaneous changes of dermatomyositis in patients with normal muscle enzymes: dermatomyositis sine myositis?","volume":"28","author":"Stonecipher","year":"1993","journal-title":"J. Am. Acad. Dermatol"},{"key":"B27","doi-asserted-by":"publisher","first-page":"1713","DOI":"10.1093\/rheumatology\/keq149","article-title":"Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis","volume":"49","author":"Takahisa","year":"2010","journal-title":"Rheumatol. Oxf."},{"key":"B28","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1186\/s13075-023-03100-z","article-title":"Mortality risk in patients with anti-MDA5 dermatomyositis is related to rapidly progressive interstitial lung disease and anti-Ro52 antibody","volume":"25","author":"Wang","year":"2023","journal-title":"Arthritis Res. and Ther."},{"key":"B29","doi-asserted-by":"publisher","first-page":"3704","DOI":"10.1093\/rheumatology\/keab928","article-title":"Intravenous immunoglobulin for interstitial lung diseases of anti-melanoma differentiation-associated gene 5-positive dermatomyositis","volume":"61","author":"Wang","year":"2022","journal-title":"Rheumatol. Oxf."},{"key":"B30","doi-asserted-by":"publisher","first-page":"356040","DOI":"10.1155\/2015\/356040","article-title":"Regulatory effect of iguratimod on the balance of Th subsets and inhibition of inflammatory cytokines in patients with rheumatoid arthritis","volume":"2015","author":"Xu","year":"2015","journal-title":"Mediat. Inflamm."},{"key":"B31","doi-asserted-by":"publisher","first-page":"e22978","DOI":"10.1002\/jcla.22978","article-title":"Serum KL-6 level is a prognostic marker in patients with anti-MDA5 antibody-positive dermatomyositis associated with interstitial lung disease","volume":"33","author":"Ye","year":"2019","journal-title":"J. Clin. Lab. Anal."}],"container-title":["Frontiers in Pharmacology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fphar.2024.1472667\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,4]],"date-time":"2024-11-04T09:14:22Z","timestamp":1730711662000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fphar.2024.1472667\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,30]]},"references-count":31,"alternative-id":["10.3389\/fphar.2024.1472667"],"URL":"https:\/\/doi.org\/10.3389\/fphar.2024.1472667","relation":{},"ISSN":["1663-9812"],"issn-type":[{"value":"1663-9812","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,10,30]]},"article-number":"1472667"}}