儿童IgG4相关疾病的管理:系统性审查
Evdoxia Sapountzi1,2, Eleni P Kotanidou2, Vasiliki-Rengina Tsinopoulou2
1Outpatient Rheumatology Unit, 2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, AHEPA University General Hospital, 54636 Thessaloniki, Greece.
Children (Basel, Switzerland)
|February 26, 2025
概括
系统性类固醇是儿童IgG4相关疾病 (IgG4-RD) 的有效一线治疗方法. 组合疗法,包括免疫抑制剂和Rituximab等生物药物,改善了多器官IgG4-RD病例的结果.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 免疫学 免疫学 免疫学
- 系统审查是系统的审查.
背景情况:
- 免疫球蛋白G4相关疾病 (IgG4-RD) 是一种复杂的多器官疾病,治疗策略各不相同.
- 儿科IgG4-RD缺乏特定的治疗算法,需要实践分析.
研究的目的:
- 系统地审查儿童IgG4相关疾病的治疗方法.
- 评估治疗结果,并在现实世界实践中确定有效的管理策略.
主要方法:
- 在PubMed和谷歌学者的系统文献搜索中,寻找儿科IgG4-RD病例 (2012-2024年).
- 手动提取81项研究 (114例) 的治疗细节,器官参与和结果.
主要成果:
- 系统性类固醇在约75%的儿科IgG4-RD病例中使用,通常与其他药物结合使用.
- 免疫抑制剂 (阿扎西奥普林,mycophenolate mofetil) 和生物药物 (rituximab) 是常见的,特别是在多器官或耐火性疾病中.
- 组合疗法显示出更高的疗效;复发发生在30%左右,特别是在老年儿童和女性中.
结论:
- 系统性类固醇是有效的儿科IgG4-RD的第一线治疗.
- 非类固醇替代品和组合疗法对于管理IgG4-RD至关重要,特别是在严重或多器官病例中.
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