在儿童中发生系统性红斑狼
Kunal Chandwar1, Amita Aggarwal2
1Department of Clinical Immunology & Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, Uttar Pradesh, India.
Indian journal of pediatrics
|September 15, 2023
概括
系统性红斑狼 (SLE) 是一种影响多个器官的自身免疫性疾病. 早期诊断和改进的治疗方法使10年生存率提高到近90%.
科学领域:
- 免疫学 免疫学 免疫学
- 类风湿病学 类风湿病学
- 儿科 儿科 儿科
背景情况:
- 系统性红斑狼 (SLE) 是一种复杂的自身免疫性疾病,影响各种器官,包括皮肤,关节和脏.
- 通过自身抗体 (ANA,anti-dsDNA,anti-RNA相关蛋白质) 的特征,SLE的发病包括免疫复合体沉积,补体激活和细胞功能障碍.
- 儿科SLE呈现出各种症状,如发烧,细胞衰竭,皮疹和器官特异性表现,需要及时评估,特别是对于脏参与.
研究的目的:
- 为儿童提供系统性红斑狼 (SLE) 的全面概述.
- 要突出诊断标准,致病机制和儿科SLE的临床表现.
- 讨论当前的治疗策略和SLE的不断变化的预后.
主要方法:
- 对SLE病变发生,临床表现和儿科患者的治疗现有文献的综述.
- 对诊断标记物的分析,包括自身抗体和器官特异性评估.
- 检查从皮质类固醇到免疫抑制剂和新兴向治疗的治疗干预措施.
主要成果:
- 儿科SLE诊断依赖于一组临床和血清学发现,其中脏和血液学参与是常见的.
- 病理机制包括免疫复杂介导的损伤,炎症性细胞因子释放 (例如,干扰素-α) 和中性粒细胞外细胞陷 (NET) 形成.
- 治疗策略是根据疾病的严重程度而定制的,在严重病例中使用氧化,mycophenolate mofetil,azathioprine,cyclophosphamide和脉冲甲基prednisolone等药物.
结论:
- 儿科SLE的预后明显改善,由于早期诊断和治疗的进步,10年生存率接近90%.
- 综合管理,包括定期随访和并发症治疗,对于尽量减少长期器官损伤和改善患者的治疗结果至关重要.
- 针对性治疗的持续研究有望进一步降低患病率,提高SLE儿童的生活质量.
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