多发性肌炎/皮肌炎相关间质性肺病治疗的研究进展

doi:10.3969/j.issn.1674-8115.2024.04.015

摘要/Abstract

摘要：

多发性肌炎/皮肌炎（polymyositis/dermatomyositis，PM/DM）是一组病因未明，以骨骼肌炎症浸润为特征的异质性自身免疫性疾病。PM/DM患者中间质性肺病（interstitial lung disease，ILD）的发病率较高，合并ILD是影响PM和DM患者预后的最重要因素。现有证据表明PM/DM相关ILD异质性大，需要结合ILD的起病形式、进展速度、病变受累范围、实验室检查、是否存在预后不良因素以及对治疗的反应等进行个体化施治。文章总结了近年来PM/DM相关ILD在治疗方面的研究进展，包括传统治疗药物的选择、用药时机、临床上在该领域尝试的新的相关用药（如生物制剂、Janus激酶抑制剂）、静脉输注免疫球蛋白、抗纤维化药物，以及潜在的有效的非药物治疗方法，旨在帮助临床医师更好地制定PM/DM-ILD治疗方案，从而改善患者的预后。

关键词: 多发性肌炎, 皮肌炎, 间质性肺病, 药物治疗

Abstract:

Polymyositis/dermatomyositis (PM/DM) is a heterogeneous autoimmune disease characterized by inflammatory cell infiltration in skeletal muscles with unknown etiology. The incidence of interstitial lung disease (ILD) in PM/DM is higher. ILD is the most important factor affecting its prognosis. Existing evidence suggests that there is significant heterogeneity in PM/DM-related ILD, which requires individualized treatment based on the onset form, progression rate, lesion involvement range, laboratory examination, presence of adverse prognostic factors, and response to treatment of ILD. This article summarizes the research progress in the treatment of PM/DM-related ILD in recent years, including the selection of traditional therapeutic drugs, timing of medication, and new drugs attempted in this field in clinical practice (such as biological agents and Janus kinase inhibitors), intravenous immunoglobulin, anti-fibrotic drugs, and potentially effective non-drug treatment methods, aiming at providing reference for clinical doctors to formulate PM/DM-ILD treatment plans and improve patient prognosis.

Key words: polymyositis, dermatomyositis, interstitial lung disease, medication

中图分类号:

R593.2

郑晓峰, 徐凌. 多发性肌炎/皮肌炎相关间质性肺病治疗的研究进展[J]. 上海交通大学学报（医学版）, 2024, 44(4): 531-536.

ZHENG Xiaofeng, XU Ling. Research progress in the treatment of interstitial lung disease related to polymyositis/dermatomyositis[J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(4): 531-536.

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