静脉免疫球蛋白有效二级预防特发性系统性毛细血管渗漏综合征的系统评价  

Effective secondary prevention of idiopathic systemic capillary leakage syndrome with intravenous immunoglobulin:a systematic review

作　　者：李燕[1] 张欣怡 唐文娇 张丽[1] 潘崚[1] 牛挺[1] LI Yan;ZHANG Xinyi;TANG Wenjiao;ZHANG Li;PAN Ling;NIU Ting(Department of Hematology,West China Hospital,Sichuan University,Chengdu 610041,P.R.China)

机构地区：[1]四川大学华西医院血液内科,成都610041

出　　处：《中国循证医学杂志》2023年第7期797-801,共5页Chinese Journal of Evidence-based Medicine

摘　　要：目的系统评价静脉免疫球蛋白(IVIG)对特发性系统性毛细血管渗漏综合征(ISCLS)患者预后的影响。方法计算机检索PubMed、CNKI和WanFang Data数据库,搜集关于IVIG预防ISCLS的个案报告和病例系列研究,检索时限均为建库至2021年12月31日。由2名研究者独立进行文献筛选、提取相关资料后,对患者预后数据进行分析。结果共纳入143篇个案报告(175例患者)和5篇病例系列(169例患者)。约75%患者有单克隆丙种球蛋白,多为IgG、Kappa型。共40例患者在病程中接受了IVIG的预防性治疗,以每月接受1次大剂量(2 g/kg)IVIG的输注为主。ISCLS患者接受IVIG二级预防治疗后5年、10年生存率分别为96%、72%,显著优于未接受IVIG组的66%、43%;接受IVIG二级预防组每年中位急性发作次数为0(0~20),未使用IVIG组为2(1~16)。结论大剂量(2g/kg)IVIG的二级预防可提高ISCLS患者的长期生存,但其在急性发作期的治疗有效性尚不明确。Objective To evaluate the effect of intravenous immunoglobulin(IVIG)on prognosis of patients with idiopathic systemic capillary leakage syndrome(ISCLS).Methods Case reports and case series related to IVIG on prognosis of ISCLS were electronicaly searched from the PubMed,CNKI and WanFang Data databases from inception to December 31,2021.Two researchers screened literature and extracted the data independently,then,prognostic data were analyzed.Results A total of 143 case reports(175 patients)and 5 case series(169 patients)were included.About 75%of patients had monoclonal gamma globulin,most of those were IgG k type.A total of 40 patients received prophylaxis with IVIG,most of whom received a high dose(2 g/kg)of IVIG per month.The 5-year and 10-year survival rates of ISCLS patients receiving IVIG secondary prevention treatment were 96%and 72%,respectively,significantly better than the rates of 66%and 43%in the group without IVIG.The median number of acute episodes per year was O(0-20)in the group receiving secondary prevention with IVIG and 2(1-16)in the group not receiving IVIG.Conclusion High-dose(2g/kg)IVIG can improve the long-term survival of ISCLS patients,but efficacy of IVIG in acute episodes is unclear.

关 键 词：系统性毛细血管渗漏综合征 Clarkson病 静脉免疫球蛋白 预防性治疗 单克隆丙种球蛋白血症 

分 类 号：R543.7[医药卫生—心血管疾病]