Yakai Fu, Zhiwei Chen, Jie Chen, Wanlong Wu, Liyang Gu, Yiyangzi Ma, Runci Wang, Kaiwen Wang, Cuiying Xie, Yi Chen, Jiayi Cai, Qiong Fu, Xiaodong Wang, Shuang Ye
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引用次数: 0
Abstract
Background: Anti-melanoma differentiation-associated gene 5-positive dermatomyositis (MDA5+ DM) with rapidly progressive interstitial lung disease (RPILD) is a fatal disease. Although Janus kinase inhibitors (JAKi) hold their promise in treating MDA5+ DM, regimen for RPILD is still urgently needed to improve the adverse prognosis.
Methods: Based on a large inception cohort of MDA5+ DM, patients with RPILD (oxygen index [OI] < 300 within the first 3 months of disease duration) were included. Patients who received protein A immunoadsorption plus a JAKi were compared to those treated with JAKi alone. Propensity score matching (PSM) was performed to adjust confounding factors. Survival analysis was conducted to evaluate the efficacy of immunoadsorption add-on.
Findings: From October 2017 to April 2024, a total of 152 newly diagnosed MDA5+ DM patients with RPILD were eligible for analyses. Thirty-four patients who underwent immunoadsorption with JAKi (ProJAK group) and 68 patients who received JAKi with or without other immunosuppressants combination (JAK group) as controls were compared after 1:2 PSM. The 6-month transplantation-free survival was significantly improved from 16.2% in the JAK group to 41.2% in the ProJAK group (p = 0.004). Subgroup analysis suggested that ProJAK therapy tended to have a more significant survival gain in patients with OI < 200. The most common adverse effect of ProJAK was transient immunoglobin decrease, but the infectious rate was similar between the 2 groups.
Conclusions: Immunoadsorption in combination with JAKi might be a promising treatment approach for MDA5+ DM patients with RPILD.
Funding: This work was supported by NFSC and SHDC.
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Med is a flagship medical journal published monthly by Cell Press, the global publisher of trusted and authoritative science journals including Cell, Cancer Cell, and Cell Reports Medicine. Our mission is to advance clinical research and practice by providing a communication forum for the publication of clinical trial results, innovative observations from longitudinal cohorts, and pioneering discoveries about disease mechanisms. The journal also encourages thought-leadership discussions among biomedical researchers, physicians, and other health scientists and stakeholders. Our goal is to improve health worldwide sustainably and ethically.
Med publishes rigorously vetted original research and cutting-edge review and perspective articles on critical health issues globally and regionally. Our research section covers clinical case reports, first-in-human studies, large-scale clinical trials, population-based studies, as well as translational research work with the potential to change the course of medical research and improve clinical practice.
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