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=Volume 12 - 2024

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, there is no established integrated treatment regimen 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 with a combination of tocilizumab and plasma exchange, along with a review of relevant literature.Methods: A literature review was conducted to gain insights into the clinical features and treatments strategies for 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 through the use of a combination of immunosuppressive agents, plasma exchange, and tocilizumab.Conclusion: Tocilizumab may serve as an effective adjunctive treatment for patients with refractory anti-MDA5 positive JDM complicated by RP-ILD who do not respond to conventional intensive immunosuppressive therapies.