Qiu Lingzhi, Shao Xiaoyan, Ma Le, Fan Zhidan, Yu Haiguo
Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China.
Front Pediatr. 2024 Nov 27;12:1497168. doi: 10.3389/fped.2024.1497168. eCollection 2024.
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.
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.
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.
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.
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。
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。
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。
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。