{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T10:23:15Z","timestamp":1787998995344,"version":"build-2784847793"},"reference-count":49,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T00:00:00Z","timestamp":1768435200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Juvenile dermatomyositis (JDM) is a rare, idiopathic autoimmune disorder characterized by inflammation of both muscle and skin, with a significant contribution from the interferon (IFN) pathway in its pathogenesis. Here, we present the case of a 4-year-old boy with JDM who tested positive for Mi2-\u03b1 and MDA5 antibodies and showed combined muscle and skin involvement. In view of his markedly elevated IFN signature, the Janus kinase (JAK) inhibitor baricitinib was introduced very early as a targeted steroid-sparing agent in addition to standard immunosuppressive therapy. The patient experienced marked clinical improvement, with resolution of skin lesions, normalization of MDA5 antibodies, and a pronounced reduction in the IFN signature. This case highlights the potential efficacy of JAK inhibition in managing JDM with a high IFN signature and supports a mechanism-based, interferon-targeted treatment approach, in line with emerging evidence in refractory JDM. Further studies are warranted to define the role of JAK inhibitors in the treatment of JDM.<\/jats:p>","DOI":"10.3390\/jcm15020709","type":"journal-article","created":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T12:31:41Z","timestamp":1768480301000},"page":"709","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Towards New Strategies: Case Report and Review of the Literature\u2014Effective Use of JAK Inhibitor Baricitinib in a 4-Year-Old Boy with Anti-MDA5 Antibody-Positive Juvenile Dermatomyositis"],"prefix":"10.3390","volume":"15","author":[{"given":"Oana","family":"Buzoianu","sequence":"first","affiliation":[{"name":"Division of Pediatric Rheumatology, Department of Pediatrics and Autoinflammation Reference Center Tuebingen, University Hospital Tuebingen, 72076 Tuebingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00d6zlem","family":"Satirer","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Department of Pediatrics and Autoinflammation Reference Center Tuebingen, University Hospital Tuebingen, 72076 Tuebingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasmin B.","family":"Kuemmerle-Deschner","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Department of Pediatrics and Autoinflammation Reference Center Tuebingen, University Hospital Tuebingen, 72076 Tuebingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6817-2798","authenticated-orcid":false,"given":"Christiane","family":"Reiser","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Department of Pediatrics and Autoinflammation Reference Center Tuebingen, University Hospital Tuebingen, 72076 Tuebingen, Germany"},{"name":"Department of Pediatrics, Landeskrankenhaus Bregenz, Carl-Pedenz-Str. 2, 6900 Bregenz, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,1,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1007\/s11926-012-0249-3","article-title":"Morbidity and mortality in adult polymyositis and dermatomyositis","volume":"14","author":"Marie","year":"2012","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_2","unstructured":"Cassidy, J.T., Petty, R.E., Laxer, R.M., and Lindsley, C.B. (2010). Textbook of Pediatric Rheumatology E-Book, Saunders\/Elsevier."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"59","DOI":"10.2119\/2006-00085.Baechler","article-title":"An interferon signature in the peripheral blood of dermatomyositis patients is associated with disease activity","volume":"13","author":"Baechler","year":"2007","journal-title":"Mol. Med."},{"key":"ref_4","first-page":"e1193","article-title":"Identification of distinctive interferon gene signatures in different types of myositis","volume":"93","author":"Derfoul","year":"2019","journal-title":"Neurology"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1625","DOI":"10.1080\/14656566.2024.2392021","article-title":"Current evidence for janus kinase inhibitors in adult and juvenile dermatomyositis and key comparisons","volume":"25","author":"Wallwork","year":"2024","journal-title":"Expert Opin. Pharmacother."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1007\/s40674-021-00182-1","article-title":"Inhibiting interferon pathways in dermatomyositis: Rationale and preliminary evidence","volume":"7","author":"Mammen","year":"2021","journal-title":"Curr. Treat. Opt. Rheumatol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1038\/s41584-021-00726-8","article-title":"Janus kinase-targeting therapies in rheumatology: A mechanisms-based approach","volume":"18","author":"Tanaka","year":"2022","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"Yang, Y., Li, Y., Yuan, W., Zhang, S., He, X., and Ji, J. (2025). Risk factors for mortality in anti-MDA5 antibody-positive dermatomyositis with interstitial lung disease: A systematic review and meta-analysis. Front. Immunol., 16.","DOI":"10.3389\/fimmu.2025.1628748"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"e24726","DOI":"10.1002\/jcla.24726","article-title":"Roles of biomarkers in anti-MDA5-positive dermatomyositis, associated interstitial lung disease, and rapidly progressive interstitial lung disease","volume":"36","author":"Li","year":"2022","journal-title":"J. Clin. Lab. Anal."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1002\/acr.20071","article-title":"Protocols for the initial treatment of moderately severe juvenile dermatomyositis: Results of a Children\u2019s Arthritis and Rheumatology Research Alliance Consensus Conference","volume":"62","author":"Huber","year":"2010","journal-title":"Arthritis Care Res."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e80","DOI":"10.1093\/brain\/awy255","article-title":"A child with severe juvenile dermatomyositis treated with ruxolitinib","volume":"141","author":"Aeschlimann","year":"2018","journal-title":"Brain"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1186\/s12969-018-0257-6","article-title":"Development of practice and consensus-based strategies including a treat-to-target approach for the management of moderate and severe juvenile dermatomyositis in Germany and Austria","volume":"16","author":"Hinze","year":"2018","journal-title":"Pediatr. Rheumatol. Online J."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1186\/s12969-023-00894-9","article-title":"Rapid and sustained response to JAK inhibition in a child with severe MDA5 + juvenile dermatomyositis","volume":"21","author":"Strauss","year":"2023","journal-title":"Pediatr. Rheumatol. Online J."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1007\/s00393-019-0643-6","article-title":"Juvenile dermatomyositis-what\u2019s new?","volume":"78","author":"Hinze","year":"2019","journal-title":"Z. Rheumatol."},{"key":"ref_15","first-page":"4732","article-title":"Approach to Janus kinase inhibition for juvenile dermatomyositis among CARRA and PReS providers","volume":"64","author":"Sherman","year":"2025","journal-title":"Rheumatology"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1609","DOI":"10.1093\/brain\/awy105","article-title":"JAK inhibitor improves type I interferon induced damage: Proof of concept in dermatomyositis","volume":"141","author":"Ladislau","year":"2018","journal-title":"Brain"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1224","DOI":"10.1111\/bjd.15006","article-title":"Correlation of cutaneous disease activity with type 1 interferon gene signature and interferon beta in dermatomyositis","volume":"176","author":"Huard","year":"2017","journal-title":"Br. J. Dermatol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"5132","DOI":"10.1093\/rheumatology\/keaf227","article-title":"Targeting interferon responses in juvenile dermatomyositis: Siglec-1 as an in vitro biomarker for JAK inhibitor efficacy","volume":"64","author":"Veldkamp","year":"2025","journal-title":"Rheumatology"},{"key":"ref_19","unstructured":"EMA (2020, January 08). Available online: https:\/\/www.ema.europa.eu\/en\/medicines\/human\/EPAR\/olumiant."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e136","DOI":"10.1093\/rheumatology\/keaa657","article-title":"Interferon signature guiding therapeutic decision making: Ruxolitinib as first-line therapy for severe juvenile dermatomyositis?","volume":"60","author":"Heinen","year":"2021","journal-title":"Rheumatology"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1227","DOI":"10.1093\/rheumatology\/keac399","article-title":"Long-term follow-up of Janus-kinase inhibitor and novel active disease biomarker in juvenile dermatomyositis","volume":"62","author":"Huang","year":"2023","journal-title":"Rheumatology"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1136\/annrheumdis-2020-218582","article-title":"Janus kinase inhibitor significantly improved rash and muscle strength in juvenile dermatomyositis","volume":"80","author":"Ding","year":"2021","journal-title":"Ann. Rheum. Dis."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1111\/1756-185X.14258","article-title":"Janus kinase inhibition in induction treatment of anti-MDA5 juvenile dermatomyositis-associated rapidly progressive interstitial lung disease","volume":"25","author":"Mopur","year":"2022","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1582","DOI":"10.1111\/1756-185X.14630","article-title":"Cardiac involvement in a case of juvenile dermatomyositis with positive anti-melanoma differentiation associated protein 5 antibody","volume":"26","author":"Kaplan","year":"2023","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_25","first-page":"e15960","article-title":"Juvenile dermatomyositis-associated calcinosis successfully treated with combined immunosuppressive, bisphosphonate, oral baricitinib and physical therapy","volume":"35","author":"Perez","year":"2022","journal-title":"Dermatol. Ther."},{"key":"ref_26","first-page":"402","article-title":"Efficacy of Janus kinase inhibitor baricitinib in the treatment of refractory juvenile dermatomyositis complicated by calcinosis","volume":"41","author":"Mastrolia","year":"2023","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"e8","DOI":"10.1093\/brain\/awz005","article-title":"Janus kinase 1\/2 inhibition with baricitinib in the treatment of juvenile dermatomyositis","volume":"142","author":"Papadopoulou","year":"2019","journal-title":"Brain"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"5801","DOI":"10.1093\/rheumatology\/keab116","article-title":"JAK inhibitors are effective in a subset of patients with juvenile dermatomyositis: A monocentric retrospective study","volume":"60","author":"Gitiaux","year":"2021","journal-title":"Rheumatology"},{"key":"ref_29","doi-asserted-by":"crossref","unstructured":"Kostik, M.M., Raupov, R.K., Suspitsin, E.N., Isupova, E.A., Gaidar, E.V., Gabrusskaya, T.V., Kaneva, M.A., Snegireva, L.S., Likhacheva, T.S., and Miulkidzhan, R.S. (2022). The Safety and Efficacy of Tofacitinib in 24 Cases of Pediatric Rheumatic Diseases: Single Centre Experience. Front. Pediatr., 10.","DOI":"10.3389\/fped.2022.820586"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1080\/24725625.2020.1832755","article-title":"Fatal outcome of anti-MDA5 juvenile dermatomyositis in a paediatric COVID-19 patient: A case report","volume":"5","author":"Remesal","year":"2021","journal-title":"Mod. Rheumatol. Case Rep."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1038\/nrd.2017.201","article-title":"JAK inhibition as a therapeutic strategy for immune and inflammatory diseases","volume":"16","author":"Schwartz","year":"2017","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1467","DOI":"10.1007\/s10067-025-07360-9","article-title":"Safety of Janus kinase inhibitors in rheumatoid arthritis: A disproportionality analysis using FAERS database from 2012 to 2022","volume":"44","author":"Mikaeili","year":"2025","journal-title":"Clin. Rheumatol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1038\/nrrheum.2017.51","article-title":"Erratum: The emerging safety profile of JAK inhibitors in rheumatic disease","volume":"13","author":"Winthrop","year":"2017","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1136\/annrheumdis-2016-209247","article-title":"Consensus-based recommendations for the management of juvenile dermatomyositis","volume":"76","author":"Baildam","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e59","DOI":"10.1093\/brain\/awz293","article-title":"Treatment of anti-MDA5 autoantibody-positive juvenile dermatomyositis using tofacitinib","volume":"142","author":"Sabbagh","year":"2019","journal-title":"Brain"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1927","DOI":"10.1093\/rheumatology\/kez525","article-title":"Anti-MDA5 juvenile idiopathic inflammatory myopathy: A specific subgroup defined by differentially enhanced interferon-alpha signalling","volume":"59","author":"Melki","year":"2020","journal-title":"Rheumatology"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"e140","DOI":"10.1093\/rheumatology\/keaa360","article-title":"A striking treatment option for recalcitrant calcinosis in juvenile dermatomyositis: Tofacitinib citrate","volume":"59","author":"Sozeri","year":"2020","journal-title":"Rheumatology"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1136\/annrheumdis-2020-218690","article-title":"Janus kinase (JAK) inhibition with baricitinib in refractory juvenile dermatomyositis","volume":"80","author":"Kim","year":"2021","journal-title":"Ann. Rheum. Dis."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1093\/mrcr\/rxac010","article-title":"Colchicine as rescue treatment in two pediatric patients with chronic recurrent multifocal osteomyelitis (CRMO)","volume":"7","author":"Lancharro","year":"2023","journal-title":"Mod. Rheumatol. Case Rep."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1186\/s12969-022-00675-w","article-title":"Progressive, refractory macrophage activation syndrome as the initial presentation of anti-MDA5 antibody positive juvenile dermatomyositis: A case report and literature review","volume":"20","author":"Stewart","year":"2022","journal-title":"Pediatr. Rheumatol. Online J."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"3579","DOI":"10.12998\/wjcc.v10.i11.3579","article-title":"Anti-nuclear matrix protein 2+ juvenile dermatomyositis with severe skin ulcer and infection: A case report and literature review","volume":"10","author":"Wang","year":"2022","journal-title":"World J. Clin. Cases"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1499","DOI":"10.1136\/ard-2023-223919","article-title":"Efficiency of tofacitinib in refractory interstitial lung disease among anti-MDA5 positive juvenile dermatomyositis patients","volume":"82","author":"Xue","year":"2023","journal-title":"Ann. Rheum. Dis."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1186\/s13075-023-03170-z","article-title":"Janus kinase inhibitor, tofacitinib, in refractory juvenile dermatomyositis: A retrospective multi-central study in China","volume":"25","author":"Zhang","year":"2023","journal-title":"Arthritis Res. Ther."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1700","DOI":"10.1093\/rheumatology\/keaa558","article-title":"Successful management with Janus kinase inhibitor tofacitinib in refractory juvenile dermatomyositis: A pilot study and literature review","volume":"60","author":"Yu","year":"2021","journal-title":"Rheumatology"},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Xiangyuan, C., Xiaoling, Z., Guangchao, S., Huasong, Z., and Dexin, L. (2024). Juvenile dermatomyositis complications: Navigating gastrointestinal perforations and treatment challenges, a case report. Front. Pediatr., 12.","DOI":"10.3389\/fped.2024.1419355"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1186\/s12969-024-01021-y","article-title":"14-month-old female with anti-MDA5 juvenile dermatomyositis complicated by liver disease: A case report","volume":"22","author":"Kinkor","year":"2024","journal-title":"Pediatr. Rheumatol. Online J."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1111\/pde.15469","article-title":"Cutaneous ulceration in juvenile dermatomyositis with anti-melanoma differentiation-associated gene 5","volume":"41","author":"Huang","year":"2024","journal-title":"Pediatr. Dermatol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"5487","DOI":"10.1093\/rheumatology\/keaf214","article-title":"Myositis-specific autoantibody subtypes are associated with response to Janus kinase inhibitors in patients with juvenile dermatomyositis","volume":"64","author":"Moreau","year":"2025","journal-title":"Rheumatology"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"e005651","DOI":"10.1136\/rmdopen-2025-005651","article-title":"Tofacitinib monotherapy as maintenance treatment in juvenile dermatomyositis: A case report","volume":"11","year":"2025","journal-title":"RMD Open"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/2\/709\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T13:28:26Z","timestamp":1768483706000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/2\/709"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,15]]},"references-count":49,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,1]]}},"alternative-id":["jcm15020709"],"URL":"https:\/\/doi.org\/10.3390\/jcm15020709","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,15]]}}}