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托珠单抗成功治疗抗MDA5阳性幼年皮肌炎相关的快速进展性间质性肺疾病:一例报告及文献综述

Successful tocilizumab treatment for rapidly progressive interstitial lung disease with anti-MDA5-positive juvenile dermatomyositis: a case report and literature review.

作者信息

Qiu Lingzhi, Shao Xiaoyan, Ma Le, Fan Zhidan, Yu Haiguo

机构信息

Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China.

出版信息

Front Pediatr. 2024 Nov 27;12:1497168. doi: 10.3389/fped.2024.1497168. eCollection 2024.

Abstract

BACKGROUND

Currently, no established integrated treatment regimen exists for anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive juvenile dermatomyositis (JDM) complicated by rapidly progressive interstitial lung disease (RP-ILD). We present a case of refractory anti-MDA5-positive JDM with RP-ILD that was successfully treated using a combination of tocilizumab and plasma exchange, along with a review of the relevant literature.

METHODS

A literature review was conducted to gain insights into the clinical features and treatment strategies for managing refractory anti-MDA5-positive JDM complicated by RP-ILD.

RESULTS

We report a case of successful management of anti-MDA5-positive JDM complicated by RP-ILD using a combination of immunosuppressive agents, plasma exchange, and tocilizumab.

CONCLUSION

Tocilizumab may serve as an effective adjunctive treatment option for patients with refractory anti-MDA5-positive JDM complicated by RP-ILD who do not respond to conventional intensive immunosuppressive therapies.

摘要

背景

目前,对于抗黑色素瘤分化相关基因5(anti-MDA5)阳性的幼年皮肌炎(JDM)合并快速进展性间质性肺病(RP-ILD),尚无既定的综合治疗方案。我们报告1例难治性anti-MDA5阳性JDM合并RP-ILD患者,其通过托珠单抗和血浆置换联合治疗获得成功,并对相关文献进行综述。

方法

进行文献综述,以深入了解难治性anti-MDA5阳性JDM合并RP-ILD的临床特征和治疗策略。

结果

我们报告1例通过免疫抑制剂、血浆置换和托珠单抗联合治疗成功管理的anti-MDA5阳性JDM合并RP-ILD患者。

结论

对于对传统强化免疫抑制治疗无反应的难治性anti-MDA5阳性JDM合并RP-ILD患者,托珠单抗可能是一种有效的辅助治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e743/11631605/c67907bb6bd4/fped-12-1497168-g001.jpg

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