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The primary effectiveness end-point was lung transplantation-free survival within 1\u2005year. Propensity score-based inverse probability of treatment weighting (IPTW) was applied for adjustment in this real-world study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In the eligible cohort, a total of 94 (32.4%) and 105 (46.7%) patients died or underwent lung transplantation within 1\u2005year in the tofacitinib group (n=290) and the CNI group (n=225), respectively. After adjustment by IPTW, patients\u2019 lung transplantation-free survival rate within 1\u2005year was significantly higher in the tofacitinib group compared to the CNI group (log-rank p=0.013). Multivariable Cox analysis performed in the IPTW dataset revealed that the hazard ratio of tofacitinib<jats:italic>versus<\/jats:italic>CNI for 1-year survival was 0.72 (95% CI 0.56\u20130.94; p=0.013). The adjusted difference of survival rate was 9.3% (95% CI 2.8\u201315.8%). Alternative analytic strategies yielded consistent results in sensitivity analyses. Patients aged &lt;60\u2005years, without rapidly progressive ILD, or with baseline arterial oxygen tension\/inspiratory oxygen fraction \u2265300\u2005mmHg might benefit more from tofacitinib. Opportunistic infection was the major treatment-related serious adverse event, with generally comparable incidence (42.4%<jats:italic>versus<\/jats:italic>45.3%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In this large multicentre cohort study, tofacitinib showed significantly more benefits for 1-year lung transplantation-free survival than calcineurin inhibitors in MDA5<jats:sup>+<\/jats:sup>DM-ILD.<\/jats:p><\/jats:sec>","DOI":"10.1183\/13993003.01488-2024","type":"journal-article","created":{"date-parts":[[2025,1,30]],"date-time":"2025-01-30T17:15:18Z","timestamp":1738257318000},"page":"2401488","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":57,"title":["Effectiveness and safety of tofacitinib<i>versus<\/i>calcineurin inhibitor in interstitial lung disease secondary to anti-MDA5-positive dermatomyositis: a multicentre cohort 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