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三联疗法未能改善抗MDA5阳性皮肌炎的预后:一项多中心纵向队列研究。

Triple-combination therapy did not improve prognosis in anti-MDA5 positive dermatomyositis: a multicentre longitudinal cohort study.

作者信息

You Hanxiao, Lv Chengyin, Xu Lingxiao, Wang Lei, Liu Ting, Yuan Fenghong, Yan Wei, Wei Hua, Wang Jiajia, Meng Deqian, Tan Wenfeng

机构信息

Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China.

Department of Rheumatology and Immunology, the Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi, Jiangsu, China.

出版信息

Clin Exp Rheumatol. 2025 Feb;43(2):251-259. doi: 10.55563/clinexprheumatol/jmpuxa. Epub 2024 Sep 24.

Abstract

OBJECTIVES

Anti-melanoma differentiation-associated gene 5-positive dermatomyositis (MDA5+ DM) is frequently linked with interstitial lung disease (ILD), especially the rapidly progressive ILD (RP-ILD). We conduct this research to evaluate the efficacy and safety of triple-combination (triple-combo) therapy consisting of high-dose corticosteroids, tacrolimus and intravenous cyclophosphamide in treating MDA5+ DM patients with ILD.

METHODS

A multicentre longitudinal cohort study involving 115 MDA5+ DM patients from the Nanjing Medical University Myositis Associated ILD (NMMI) cohort was conducted between January 2019 and November 2022. Patients were categorised into triple-combo and non-triple therapy groups, and their outcomes were assessed.

RESULTS

Contrary to expectations, triple-combo therapy did not improve the prognosis for MDA5+ DM patients but was linked to increased mortality rates, especially among those at high risk for RP-ILD.

CONCLUSIONS

Our study suggests that triple-combo therapy might not be effective in improving prognosis in MDA5+ DM patients. Further research is needed to establish safer and more effective treatment modalities for this patient population.

摘要

目的

抗黑色素瘤分化相关基因5阳性皮肌炎(MDA5+ DM)常与间质性肺疾病(ILD)相关,尤其是快速进展性ILD(RP-ILD)。我们开展本研究以评估由高剂量皮质类固醇、他克莫司和静脉注射环磷酰胺组成的三联疗法治疗合并ILD的MDA5+ DM患者的疗效和安全性。

方法

2019年1月至2022年11月期间,对来自南京医科大学肌炎相关ILD(NMMI)队列的115例MDA5+ DM患者进行了一项多中心纵向队列研究。将患者分为三联疗法组和非三联疗法组,并评估其结局。

结果

与预期相反,三联疗法并未改善MDA5+ DM患者的预后,反而与死亡率增加相关,尤其是在RP-ILD高危患者中。

结论

我们的研究表明,三联疗法可能无法有效改善MDA5+ DM患者的预后。需要进一步研究为该患者群体建立更安全、更有效的治疗方式。

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