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Fifteen patients (14 refractory, 1 severe newly diagnosed; all below recommended baricitinib dose) received low-dose baricitinib with corticosteroids and/or immunosuppressive agents. Skin involvement improved across the cohort; complete responses occurred within 4–12 weeks. Interstitial lung disease showed varied outcomes, and one macrophage activation syndrome case reached remission. Steroid dose decreased significantly at 24 weeks; muscle improvement was not statistically different, and adverse events were recorded.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/juvenile-dermatomyositis-low-dose-baricitinib-real-world-efficacy-and-safety-in-refractory-or-severe-pediatric-patients/497722/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/juvenile-dermatomyositis-low-dose-baricitinib-real-world-efficacy-and-safety-in-refractory-or-severe-pediatric-patients/497722.png","ImageObject",300,407,{"name":92,"@type":93},"Đào","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-11-08","2026-10-02",true,{"@type":102,"interactionType":103,"userInteractionCount":39},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What was the study objective of using low-dose baricitinib in juvenile dermatomyositis?","Question",{"text":112,"@type":113},"To evaluate the efficacy and safety of low dose baricitinib in children with refractory or severe JDM in a real-world setting.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How were treatment responses assessed after baricitinib initiation?",{"text":117,"@type":113},"Clinical data were collected at baseline and at 4, 12, and 24 weeks, and responses were evaluated using PRINTO remission criteria and skin Disease Activity Score (DAS).",{"name":119,"@type":110,"acceptedAnswer":120},"What safety findings and adverse events were reported in the study?",{"text":121,"@type":113},"All adverse events were recorded. One patient discontinued baricitinib due to varicella zoster virus infection, and no other serious side effects were observed in this study.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},497722,1791099631,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":39,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":52,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144,"preview_page_count":4},1374402968488,"https://ap-avatar.wpscdn.com/davatar_29158cc5080c5b710cf443261637dec0","Zhejiang University School of Medicine Children's Hospital [https://orcid.org/0000-0002-6645-1993](https://orcid.org/0000-0002-6645-1993)  \nZhejiang University School of Medicine Children's Hospital  \nZhejiang University School of Medicine Children's Hospital  \n 􀂦   \nThe Children's Hospital of Zhejiang University School of Medicine [https://orcid.org/0000-0002-4930-9493](https://orcid.org/0000-0002-4930-9493)  \nJuvenile dermatomyositis, Refractory, Baricitinib, Treatment.  \n January 10th, 2022  \n [https://doi.org/10.21203/rs.3.rs-1226720/v1](https://doi.org/10.21203/rs.3.rs-1226720/v1)  \n 􀁱 􀅏 This work is licensed under a Creative Commons Attribution 4.0 International License. Read Full License  \nTo evaluate the e¨cacy and safety of low dose baricitinib in children with refractory or severe juvenile dermatomyositis (JDM) in a real-world setting.  \nA monocentric retrospective real-world study was conducted, in which fourteen refractory and one severe newly diagnosed JDM patients were included. These patients were all treated by low dose baricitinib (below the recommended dose) combined with corticosteroids and or immunosuppressive agents. Clinical data were collected at the baseline and 4, 12, 24 weeks after baricitinib implication. Treatment response (complete response (CR), Partial response (PR) and non-response (NR)) was evaluated using both the Paediatric Rheumatology International Trials Organization (PRINTO) remission criteria and skin Disease Activity Score (DAS) . All the adverse events (AEs) were recorded.  \nAfter baricitinib treatment, all 15 patients showed improvement of skin involvement, including 14 patients with recurrent skin rashes and one newly diagnosed JDM. Calcinosis stabilized in two patients (2/3) and partially regressed in one. Four patients (4/15) had interstitial lung disease (ILD), which normalized in one, improved in two and stabilized in one. One patient complicated with macrophage activation syndrome (MAS) achieved clinical remission. CR was achieved in 3/15 patients, ranging from 4 to 12 weeks after baricitinib initiation. Five patients (5/15) got PR 4 to 24 weeks after baricitinib use. Daily steroid dosage was decreased from 0.632 mg/kg to 0.357 mg/kg (P = 0 .043) at 24 weeks in all responders. However, there was no statistically difference in muscle improvement. One patient was stopped using baricitinib because of varicella zoster virus infection, while no other serious side effect was observed in this study.  \nLow dose baricitinib had e¨cacy and was safe to applied in refractory or severe JDM patients, especially for recurrent skin rashes. Baricitinib may also be helpful for JDM complicated with ILD and MAS.  \nJuvenile dermatomyositis (JDM) is an autoimmune disease characterized by in§ammation of both the skin and muscles. Typical skin signs were Gottron papules, heliotrope rashes and nailfold capillary changes. Other organs may also be involved in JDM, including lungs, heart and gastrointestinal tract, leading to poor outcome of this disease. The pathogenesis of this disease is poorly understood which constraints targeted therapy drugs applying in JDM. Although the prognosis of JDM has been remarkably improved by corticosteroids (CS) combine with disease modifying antirheumatic drugs (DMARDs), there still have 20% patients who become refractory JDM or even unresponsive to the above therapy[1]. Other therapies such as intravenous immunoglobulin, rituximab, etanercept and ada limumab have yielded some promising results[2–5], but they are only bene¦cial in some of the refractory cases. Persistent skin lesions and calcinosis remain as the treatment challenge to rheumatologists. Recent studies have shown that interferons (IFNs) play a central role in the pathogenesis of both juvenile and adult dermatomyositis[6, 7]. Elevated IFN-response gene signature has been found in the blood, muscle and skin of dermatomyositis patients[8–10]. In treatment naive JDM patients, serum IFN-α activity was positively","cbCainAmVKI0BSjp","https://ap.wps.com/l/cbCainAmVKI0BSjp","pdf",1148877,"English","# Study Objective\n# Methods\n## Patient cohort and treatment protocol\n## Outcomes and follow-up schedule\n# Results\n## Skin response and disease activity\n## Interstitial lung disease and macrophage activation syndrome\n## Adverse events and safety\n## Steroid reduction and muscle outcomes\n# Discussion and Conclusions","[{\"question\":\"What was the study objective of using low-dose baricitinib in juvenile dermatomyositis?\",\"answer\":\"To evaluate the efficacy and safety of low dose baricitinib in children with refractory or severe JDM in a real-world setting.\"},{\"question\":\"How were treatment responses assessed after baricitinib initiation?\",\"answer\":\"Clinical data were collected at baseline and at 4, 12, and 24 weeks, and responses were evaluated using PRINTO remission criteria and skin Disease Activity Score (DAS).\"},{\"question\":\"What safety findings and adverse events were reported in the study?\",\"answer\":\"All adverse events were recorded. One patient discontinued baricitinib due to varicella zoster virus infection, and no other serious side effects were observed in this study.\"}]","Juvenile Dermatomyositis - Low Dose Baricitinib Real-World Efficacy and Safety in Refractory or Severe Pediatric Patients | PDF",1790965322,25]