系统性青年异常性关节炎相关的肺病:一个回顾性队列研究
Konstantin E Belozerov1,2, Natalia M Solomatina1, Eugenia A Isupova1
1Department of Pediatric, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia.
间歇性肺病 (ILD) 是儿童系统性青少年关节炎 (sJIA) 的严重并发症. 关键预测因素包括广泛的皮疹,血清炎和巨细胞激活综合征 (MAS).
科学领域:
- 儿科风湿病学 儿科风湿病学
- 肺部病理学 肺部病理学
- 免疫学 免疫学 免疫学
背景情况:
- 系统性青少年关节炎 (sJIA) 可以导致严重的肺损伤,间歇性肺病 (ILD) 是一种罕见但严重的并发症.
- 现有的关于sJIA中ILD的数据有限,报告的病例不到100例,强调需要更详细的信息.
研究的目的:
- 综合描述患有sJIA和并发性ILD的儿童的临床特征和结果.
- 确定与SJIA患者ILD发展相关的潜在预测因子和特征.
主要方法:
- 一项回顾性队列研究包括5名被诊断患有sJIA和ILD的儿科患者.
- sJIA诊断遵循了2004年和2019年国际风湿病学协会联盟的标准.
- 胸部CT证实了ILD,排除了其他原因;巨细胞激活综合征 (MAS) 使用HLH-2004和2016年EULAR/ACR/PRINTO标准进行诊断.
主要成果:
- 患者呈现系统性特征,突出的皮疹 (100%),严重的MAS (hScore 194-220),透膜炎 (100%) 和呼吸系统症状 (100%).
- 所有患者都患有多流 (100%),40%患有肺动脉高血压,60%患有托西利祖马布输液反应.
- 一名患有21型三综合症的患者死亡;75%的患者的肺部疾病有所改善,尽管一个患有持续性SJIA的患者经历了ILD进展.
结论:
- ILD代表了sJIA的危及生命的并发症,影响了不同年龄段的儿童.
- 预测ILD的预测因素包括广泛的皮疹,血清炎,MAS,淋巴缺血症,三症21和生物输液反应.
- 进一步的研究至关重要,以阐明ILD病原性,确定预测生物标志物,并为sJIA相关的ILD制定有针对性的治疗策略.
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