肺部参与青少年无病性炎症性肌肉病:一个系统的审查
Sarah Abu-Rumeileh1, Edoardo Marrani1, Valerio Maniscalco1
1Rheumatology Unit, ERN-ReCONNET center, Meyer Children Hospital IRCCS, Florence, Italy.
Autoimmunity reviews
|August 23, 2023
概括
青春期异常性炎症性肌肉病可以导致严重的肺部疾病 (ILD). 抗黑色素瘤分化相关基因5 (MDA-5) 与快速进展的ILD有关,需要早期检测和治疗以获得更好的结果.
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 儿科 儿科 儿科
背景情况:
- 青春期异常性炎症性肌肉病变 (JIIM) 是一种罕见的结缔组织疾病,具有潜在的系统性参与,包括间歇性肺病 (ILD).
- 与JIIM相关的ILD数据很少,需要对其特征进行全面审查.
结论:
- 在JIIM中,MDA-5与快速进展的ILD有关.
- ILD是一种严重的,罕见的JIIM并发症,需要迅速识别和积极管理.
- 男性性别,快速进展的疾病,以及高KL-6水平与ICU入院和死亡率相关.
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