{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T22:20:32Z","timestamp":1788992432231,"version":"build-2803163510"},"reference-count":23,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,11,27]],"date-time":"2025-11-27T00:00:00Z","timestamp":1764201600000},"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. Immunol."],"abstract":"<jats:p>A 55-year-old woman was diagnosed with anti-melanoma differentiation-associated gene 5 (anti-MDA5) positive dermatomyositis (DM) in 2020, having low grade fever, weight loss, arthritis in small joint of hands, erythematous-desquamative lesions on hands, cuticle dystrophy and severe skin ulcerations. Firstly, she was treated with cyclosporine (CsA), soon discontinued due to gastrointestinal intolerance. She was subsequently treated with steroid pulses, hydroxychloroquine (HCQ) and mycophenolate (MMF), without improvement. In March 2021 she started therapy with intravenous immunoglobulins (IVIG) and prostanoids, leading to ulcer improvement, but stopped due to gastrointestinal intolerance. A chest high resolution computed tomography (HRCT) done in June 2021 showed interstitial lung disease (ILD). In September 2021 rituximab (RTX) was stopped at the first infusion due to gastrointestinal intolerance. From January 2022 the patient also started to walk with difficulty due to development of deep asthenia. Therapy with various Jak inhibitors was started (first tofacitinib, then baricitinib, finally upadacitinib), leading to improvement of cutaneous ulcers, but stopped every time after a few months due to gastrointestinal intolerance and dizziness. In August 2023 during hospitalization a spirometry showed reduction of diffusion capacity of the lung for the carbon monoxide (DLCO). In June 2024, in consideration of poor disease control and refractoriness of the disease (loss of appetite and weight, worsening of asthenia which forced the patient into a wheelchair, persistence of polyarthritis, skin ulcers, alopecia, Gottron\u2019s signs, radiological progression of ILD), she was hospitalized again and Anifrolumab (ANI) was started in July 2024 (300 mg IV every four weeks). After four infusions the patient reported improved appetite with significant weight gain, resolution of arthritis and disappearance of cutaneous ulcers, Gottron\u2019s sign and alopecia. In February 2025, after seven ANI infusions, a HRCT demonstrated a significant radiological improvement of the ILD compared to 2024, and spirometry showed significant improvement of DLCO compared to 2023. In this period, no adverse effects were observed from the new therapy. After twelve total infusions, constitutional, articular and cutaneous involvement remained in good control. This case suggests the potential efficacy of ANI in refractory anti-MDA5-positive DM not only on skin manifestation, but also on articular and lung involvement.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1717766","type":"journal-article","created":{"date-parts":[[2025,11,27]],"date-time":"2025-11-27T06:30:23Z","timestamp":1764225023000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Efficacy of anifrolumab on skin, joint and lung involvement in anti-MDA5 positive dermatomyositis: a case report"],"prefix":"10.3389","volume":"16","author":[{"given":"Federico","family":"Pettorossi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Brischigliaro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisol","family":"Bracalenti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurora","family":"Di Gregorio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beatrice","family":"Moccaldi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Depascale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margherita","family":"Zen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabetta","family":"Zanatta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariele","family":"Gatto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Doria","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Iaccarino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,11,27]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1038\/s41572-021-00321-x","article-title":"Idiopathic inflammatory myopathies","volume":"7","author":"Lundberg","year":"2021","journal-title":"Nat Rev Dis Prime"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.3390\/diagnostics13193080","article-title":"Detection of myositis autoantibodies by multi-analytic immunoassays in a large multicenter cohort of patients with definite idiopathic inflammatory myopathies","volume":"13","author":"Ghirardello","year":"2023","journal-title":"Diagn (Basel)"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1097\/BOR.0000000000000908","article-title":"Anti-MDA5 dermatomyositis: an update from bench to bedside","volume":"34","author":"Fuzzi","year":"2022","journal-title":"Curr Opin Rheumatol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"e003121","DOI":"10.1136\/rmdopen-2023-003121","article-title":"Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: data from a large multicentric cohort","volume":"9","author":"Zanatta","year":"2023","journal-title":"RMD Open"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2023.103334","article-title":"The interferon in idiopathic inflammatory myopathies: Different signatures and new therapeutic perspectives","volume":"22","author":"Gasparotto","year":"2023","journal-title":"A Literat Rev Autoimmun Rev"},{"key":"B6","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":"B7","doi-asserted-by":"publisher","DOI":"10.55563\/clinexprheumatol\/ubov8b","article-title":"Evaluation of biomarkers related to endothelial dysfunction: proof of vasculopathy in anti-melanoma differentiation-associated gene 5 dermatomyositis","volume":"39","author":"He","year":"2021","journal-title":"Clin Exp Rheumatol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kez185","article-title":"Successful classification of macrophage-mannose receptor CD206 in severity of anti-MDA5 antibody positive dermatomyositis associated ILD","volume":"58","author":"Horiike","year":"2019","journal-title":"Rheumatol (Oxford)"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-024-07079-z","article-title":"Effectiveness and safety of low dose Rituximab as remission-maintenance treatment for patients with refractory idiopathic inflammatory myopathies: results of a retrospective study from a monocentric cohort","volume":"43","author":"Gamba","year":"2024","journal-title":"Clin Rheumatol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1007\/s12026-013-8408-9","article-title":"Rituximab in refractory idiopathic inflammatory myopathies and antisynthetase syndrome: personal experience and review of the literature","volume":"56","author":"Nalotto","year":"2013","journal-title":"Immunol Res"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.3390\/medicina57040347","article-title":"Management of myositis-associated interstitial lung disease","volume":"57","author":"Fujisawa","year":"2021","journal-title":"Med (Kaunas)"},{"key":"B12","doi-asserted-by":"publisher","first-page":"e15401","DOI":"10.1111\/1756-185X.15401","article-title":"Advance in management of anti-MDA5 antibody-positive dermatomyositis: Conquering therapeutic obstacles","volume":"27","author":"Shih","year":"2024","journal-title":"Int J Rheum Dis"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2023.04.022","article-title":"The regulatory landscape of macrophage interferon signaling in inflammation","volume":"152","author":"Siebeler","year":"2023","journal-title":"J Allergy Clin Immunol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdcr.2023.10.023","article-title":"Refractory dermatomyositis responsive to anifrolumab","volume":"43","author":"Ang","year":"2023","journal-title":"JAAD Case Rep"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2023.4744","article-title":"Rapid improvement in recalcitrant cutaneous juvenile dermatomyositis with anifrolumab treatment","volume":"160","author":"Shaw","year":"2024","journal-title":"JAMA Dermatol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2024.07.1491","article-title":"Anifrolumab in recalcitrant cutaneous dermatomyositis: A multicenter retrospective cohort study","volume":"91","author":"Shaw","year":"2024","journal-title":"J Am Acad Dermatol"},{"key":"B17","doi-asserted-by":"publisher","first-page":"e66704","DOI":"10.7759\/cureus.66704","article-title":"A Novel Treatment for Skin Manifestations in Dermatomyositis: A Case Report","volume":"16","author":"Srinivasa Murthy","year":"2024","journal-title":"Cureus"},{"key":"B18","doi-asserted-by":"publisher","first-page":"e11792","DOI":"10.1002\/acr2.11792","article-title":"Recalcitrant dermatomyositis treated with anifrolumab","volume":"7","author":"Chaudhary","year":"2025","journal-title":"ACR Open Rheumatol"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1093\/bjd\/ljaf064","article-title":"Improvement in dermatomyositis-associated muscle disease with anifrolumab","volume":"192","author":"Shayegan","year":"2025","journal-title":"Br J Dermatol"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.55563\/clinexprheumatol\/haftre","article-title":"Successful therapy of refractory dermatomyositis skin rash with anifrolumab","author":"Galluzzo","year":"2025","journal-title":"Clin Exp Rheumatol"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1111\/ijd.17675","article-title":"Successful treatment of refractory cutaneous dermatomyositis with anifrolumab","volume":"64","author":"Helm","year":"2025","journal-title":"Int J Dermatol"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1093\/ced\/llae476","article-title":"Anifrolumab use in refractory dermatomyositis: a case report and literature review","volume":"50","author":"Claudio-Oliva","year":"2025","journal-title":"Clin Exp Dermatol"},{"key":"B23","unstructured":"A multicenter, parallel-group, double-blind, 2-arm, phase III study to investigate the efficacy and safety of anifrolumab administered as subcutaneous injection and added to standard of care compared with placebo added to standard of care in adult participants with idiopathic inflammatory myopathies (Polymyositis and dermatomyositis)\n          \n          2025"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1717766\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,27]],"date-time":"2025-11-27T06:30:26Z","timestamp":1764225026000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1717766\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,27]]},"references-count":23,"alternative-id":["10.3389\/fimmu.2025.1717766"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2025.1717766","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11,27]]},"article-number":"1717766"}}