AUTHOR=Qiu Lingzhi , Shao Xiaoyan , Ma Le , Fan Zhidan , Yu Haiguo TITLE=Successful tocilizumab treatment for rapidly progressive interstitial lung disease with anti-MDA5-positive juvenile dermatomyositis: a case report and literature review JOURNAL=Frontiers in Pediatrics VOLUME=12 YEAR=2024 URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2024.1497168 DOI=10.3389/fped.2024.1497168 ISSN=2296-2360 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.