医学
青少年皮肌炎
皮肌炎
临床实习
疾病
少年
梅德林
重症监护医学
皮肤病科
家庭医学
内科学
生物
政治学
法学
遗传学
作者
Saskia R. Veldkamp,Femke van Wijk,Annet van Royen‐Kerkhof,Marc H.A. Jansen
标识
DOI:10.1016/j.berh.2024.101976
摘要
Juvenile dermatomyositis is characterized by childhood-onset chronic inflammation of the muscles and skin, with potential involvement of other organs. Patients are at risk for long-term morbidity due to insufficient disease control and steroid-related toxicity. Personalised treatment is challenged by a lack of validated tools that can reliably predict treatment response and monitor ongoing (subclinical) inflammation, and by a lack of evidence regarding the best choice of medication for individual patients. A better understanding of the involved disease mechanisms could reveal potential biomarkers and novel therapeutic targets. In this review, we highlight the most relevant immune and non-immune mechanisms, elucidating the effects of interferon overexpression on tissue alongside the interplay between the interferon signature, mitochondrial function, and immune cells. We review mechanism-based biomarkers that are promising for clinical implementation, and the latest advances in targeted therapy development. Finally, we discuss key steps needed for translating these discoveries into clinical practice.
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