Case report: Exploring efficacy of tofacitinib in modulating interferon response in five case of anti-MDA5+dermatomyositis with interstitial lung disease

被引：0
作者：
Zhao, Jie [1 ]
Bao, Yan [1 ]
Gao, Ying [1 ]
Xie, Keliang [1 ]
机构：
[1] Tianjin Med Univ, Dept Crit Care Unit, Gen Hosp, Tianjin, Peoples R China
来源：
FRONTIERS IN IMMUNOLOGY | 2025年 / 16卷
基金：
中国国家自然科学基金;
关键词：
MDA5-DM; RP-ILD; tofacitinib; interferon; cytokine; ANTIBODY; PHENOTYPES;
D O I：
10.3389/fimmu.2025.1515602
中图分类号：
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号：
100102 ;
摘要：
A case report highlights the challenges faced in managing a 66-year-old Chinese woman diagnosed with anti-MDA5 antibody-positive dermatomyositis (MDA5-DM) complicated by rapidly progressive interstitial lung disease (RP-ILD). Despite aggressive therapeutic interventions, her condition rapidly deteriorated, emphasizing the severity and devastating nature of this subtype of DM. A salient feature of her clinical presentation was the marked elevation of interferon (IFN)-gamma and IFN-alpha levels, underscoring the pivotal role that IFNs play in driving the pathogenesis and progression of MDA5-DM-related RP-ILD. In an attempt to stem the relentless progression, tofacitinib, a Janus kinase (JAK) inhibitor, was applied into her treatment regimen. This therapeutic intervention led to a transient decrease in IFN-related cytokines, offering a glimpse of hope that JAK inhibition could modulate the exaggerated IFN response implicated in the disease. Other four similar cases underscore the critical importance of early and aggressive intervention in MDA5-DM patients, as well as the potential therapeutic avenues involving IFN blockers by JAK inhibitors. Urgent and well-designed clinical trials are imperative to unravel the intricate interplay between RP-ILD and the IFN signature in MDA5-DM, and to evaluate novel therapeutic targets that promise long-term efficacy and safety.
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页数：9
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