临床特征和儿童发病的狼介质血管炎的预后作为最初的呈现 - 一个病例控制研究的研究
Jia Zhu1, Jianming Lai1, Xiaohui Liu2
1Department of Rheumatology and Immunology, Children's Hospital, Capital Institute of Pediatrics. , NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China.
Arthritis research & therapy
|December 21, 2023
概括
童年开始的全身性红斑狼 (cSLE) 与初始狼介质血管炎 (LMV) 呈现是罕见的. 这种情况虽然可以用甲基prednisolone和cyclophosphamide治疗,但导致整体恢复比非LMVcSLE差.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 胃肠病学 胃肠病学
- 血管医学 血管医学
背景情况:
- 狼中肠道血管炎 (LMV) 是儿童开始的系统性红斑狼 (cSLE) 的罕见但关键并发症.
- 关于cSLE最初呈现LMV的研究有限,特别是在中国人群中.
- 这项研究旨在对中国儿童的cSLE与初始LMV进行表征.
研究的目的:
- 分析cSLE的临床特征与最初的LMV.
- 为了评估cSLE的预后与最初的LMV.
- 在中国人口中,将cSLE与LMV与cSLE没有LMV进行比较.
主要方法:
- 一项回顾性病例控制研究包括55名cSLE患者 (2018年7月至2021年7月).
- 收集了临床,实验室,成像,治疗和随访数据,并在LMV (n=11) 和非LMV (n=44) 组之间进行了比较.
- 非LMV患者的年龄和性别与LMV患者相匹配.
主要成果:
- 在12-13岁的女孩中出现了高cSLE活性的LMV;腹痛是最常见的.
- 与非LMV患者相比,LMV患者的胃肠道症状,血清腔,脏和肺部损伤率更高.
- 在LMV患者中,腹部CT显示了更高的肠壁加厚,腹输液和中肠血管增强.
结论:
- 在cSLE中,LMV与高的疾病活性有关,并影响多个器官,特别是脏,血清腔和肺.
- 甲基普雷迪尼索隆脉冲与环胺脉冲疗法相结合,对cSLE中的LMV有效.
- 虽然LMV患者的症状恢复良好,但他们的整体预后比非LMVcSLE患者更差.
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