{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T05:15:24Z","timestamp":1783401324682,"version":"3.54.6"},"reference-count":43,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2026,5,30]],"date-time":"2026-05-30T00:00:00Z","timestamp":1780099200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,5,30]],"date-time":"2026-05-30T00:00:00Z","timestamp":1780099200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Rheumatol"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction\/objective<\/jats:title>\n                    <jats:p>Janus kinase inhibitors (JAKi\u2019s) have shown promising results in systemic sclerosis (SSc), yet little studied. We evaluated safety and intra-patient changes in pulmonary, articular, and cutaneous parameters in SSc JAKi-treated patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>An international, multi-centre, longitudinal retrospective cohort study. We assessed changes in FVC%, DLCO%, modified Rodnan skin score (mRSS), tender\/swollen joint counts (TJC\/SJC), digital ulcers (DU), and calcinosis. Baseline was defined as JAKi initiation. Outcomes were analysed as delta from baseline using Wilcoxon signed-rank tests, with effect sizes expressed as standardized mean change (SMC).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Among 32 patients treated with the four available JAKi\u2019s, median (IQR) follow-up was 16.9 (10.3, 31.8) months, totalling 52.8 patient-years. At 12\u00a0months, pulmonary function remained stable (SMC\u2009=\u2009\u2009+\u20090.22 for FVC%,\u2009+\u20090.23 for DLCO%;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.10 and\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.33, respectively). mRSS (SMC\u2009=\u2009\u2009\u2212\u20090.29,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.03), TJC and SJC (SMC\u2009=\u2009\u2009\u2212\u20091.19 and\u2009\u2212\u20090.69,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001 and\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.001, respectively) significantly improved. At 24\u00a0months, numerical improvements in mRSS, TJC, and SJC persisted with SMC of\u2009\u2212\u20090.21,\u2009\u2212\u20091.13, and\u2009\u2212\u20091.14 (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.17,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.054,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.058, respectively). Among 11 patients with baseline calcinosis, 45.5% improved. Ten patients developed DUs. In 15 patients receiving glucocorticoids, a non-significant trend toward tapering was observed.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>JAKi treatment in SSc patients was associated with \u2018real-world\u2019 improvements in multiple domains; findings are exploratory.<\/jats:p>\n                    <jats:p>\n                      <jats:table-wrap>\n                        <jats:table>\n                          <jats:tbody>\n                            <jats:tr>\n                              <jats:td align=\"left\" colspan=\"2\">\n                                <jats:bold>Key Points<\/jats:bold>\n                                \u2022\n                                <jats:italic>JAKi treatment in SSc patients was associated with \u2018real-world\u2019 improvements in multiple clinically important domains.<\/jats:italic>\n                                \u2022\n                                <jats:italic>Relevant safety concerns with JAKi treatment were confirmed in our treated SSc patient cohort.<\/jats:italic>\n                                \u2022\n                                <jats:italic>Our findings provide preliminary real-world evidence supporting a potential role of JAK inhibitors across multiple clinical domains in SSc.<\/jats:italic>\n                                \u2022\n                                <jats:italic>Long-term controlled trials to confirm the safety and efficacy of JAKi\u2019s in patients with SSc are needed.<\/jats:italic>\n                              <\/jats:td>\n                            <\/jats:tr>\n                          <\/jats:tbody>\n                        <\/jats:table>\n                      <\/jats:table-wrap>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s10067-026-08168-x","type":"journal-article","created":{"date-parts":[[2026,5,30]],"date-time":"2026-05-30T09:40:07Z","timestamp":1780134007000},"page":"4335-4344","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Longitudinal clinical response to Janus kinase inhibitors in systemic sclerosis: a real-life multicentric study across multiple clinical domains"],"prefix":"10.1007","volume":"45","author":[{"given":"Stefano","family":"Di Donato","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan Jos\u00e9","family":"Alegre-Sancho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anastas","family":"Batalov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zguro","family":"Batalov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Bellando-Randone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmela","family":"Coccia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"De Pinto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dilia","family":"Giuggioli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3361-4909","authenticated-orcid":false,"given":"Michael","family":"Hughes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,5,30]]},"reference":[{"issue":"1","key":"8168_CR1","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1136\/annrheumdis-2014-205295","volume":"75","author":"J Avouac","year":"2016","unstructured":"Avouac J, Walker UA, Hachulla E et al (2016) Joint and tendon involvement predict disease progression in systemic sclerosis: a EUSTAR prospective study. 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Silvia Bellando Randone: Speaker fees and Consultancy fees from Boehringer Ingelheim. None of the other authors report any disclosures relevant to this manuscript.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}