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Objective: To compare the effectiveness and safety of tofacitinib (TOF) versus calcineurin inhibitor (CNI) as initial immunosuppressive regimen for anti-melanoma differentiation-associated gene 5-positive dermatomyositis with interstitial lung disease (MDA5+DM-ILD)., Methods: Adult Chinese patients with newly-diagnosed MDA5+DM-ILD (ILD course<3 months) from five tertiary referral centres between April 2014 and January 2023 were included for this retrospective cohort study. The primary effectiveness endpoint was lung transplantation-free survival within 1 year. Propensity score-based inverse probability of treatment weighting (IPTW) was applied for adjustment in this real-world study., Results: In the eligible cohort, a total of 94 (32.4%) and 105 (46.7%) patients died or underwent lung transplantation within 1 year in the TOF group (n=290) and the CNI group (n=225), respectively. After adjustment by IPTW, patients' lung transplantation-free survival rate within 1 year was significantly higher in the TOF group compared to the CNI group (log-rank p=0.013). Multivariable Cox analysis performed in the IPTW dataset revealed the hazard ratio of TOF versus CNI for 1-year survival was 0.72 (95% CI, 0.56 to 0.94, p=0.013). The adjusted difference of survival rate was 9.3% (95%CI 2.8% to 15.8%). Alternative analytic strategies yielded consistent results in sensitivity analyses. Patients less than 60 years old, without RPILD, or with baseline PaO 2 /FiO 2 ≥300 mmHg might benefit more from TOF. Opportunistic infection was the major treatment-related serious adverse event, with generally comparable incidence (42.4% versus 45.3%)., Conclusion: In this large multi-centre cohort study, tofacitinib showed significantly more benefits for 1-year lung transplantation-free survival than calcineurin inhibitors in MDA5+DM-ILD., (Copyright ©The authors 2025.)

Background: Systemic rheumatic diseases (SRDs), particularly anti-melanoma differentiation-associated protein 5 positive dermatomyositis (MDA5 + DM), often affect the respiratory system and have a predisposition for developing into acute respiratory failure (ARF). Venovenous extracorporeal membrane oxygenation (VV-ECMO) can provide full respiratory support and can be used as a life-saving intervention. The present study describes the clinical profiles and prognoses of patients with MDA5 + DM and other SRDs receiving VV-ECMO for ARF., Methods: A single-center retrospective study of patients with SRD who received VV-ECMO between June 2017 and February 2022 was conducted. Demographic and laboratory data, treatments, extracorporeal membrane oxygenation (ECMO) parameters, and clinical outcomes were extracted from electronic medical records and compared between patients with MDA5 + DM and other SRDs., Results: Seven patients with MDA5 + DM and four patients with other SRDs were included in the study. Treatment by ECMO was provided for 152 days. Only one patient experienced ECMO-related complications. Three patients in the other SRD group survived to ECMO decannulation, and two of these patients survived to discharge. However, no patients in the MDA5 + DM group survived to decannulation or discharge., Conclusions: Treatment by VV-ECMO could be safely applied to patients with SRDs to maintain normal respiration and oxygenation. However, patients with MDA5 + DM associated with ARF who underwent VV-ECMO had worse outcomes., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jtd.amegroups.com/article/view/10.21037/jtd-24-650/coif). The authors have no conflicts of interest to declare., (2024 AME Publishing Company. All rights reserved.)