中国血液净化

中国血液净化 ›› 2020, Vol. 19 ›› Issue (09): 623-626.doi: 10.3969/j.issn.1671-4091.2020.09.012

• 临床研究 • 上一篇    下一篇

血浆置换治疗儿童抗中性粒细胞胞浆抗体相关性血管炎的疗效观察

寻劢1,李志辉1,段翠蓉1   

  1. 1湖南省儿童医院肾脏风湿科
  • 收稿日期:2020-04-26 修回日期:2020-07-10 出版日期:2020-09-12 发布日期:2020-09-03
  • 通讯作者: 李志辉 Lizh0731@aliyun.com E-mail:Lizh0731@aliyun.com
  • 基金资助:
    湖南省儿童急性肾损伤临床医学研究中心资助[湖南省科技创新平台与人才计划(编号：2016SK4004)]

Effect of plasma exchange on anti-neutrophil cytoplasmic autoantibody-associated vasculitis in children

  1. 1Department of Nephrology and Rheumatology, Hunan Children's Hospital, Changsha 410007, China
  • Received:2020-04-26 Revised:2020-07-10 Online:2020-09-12 Published:2020-09-03

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摘要： 【摘要】目的观察血浆置换治疗儿童抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic autoantibody, ANCA)相关性血管炎的疗效。方法将2017年1月～2020年1月在湖南省儿童医院肾脏风湿科住院的诊断为ANCA 相关性血管炎的13 例患儿分为2组，对照组(n=7)单纯给予激素加免疫抑制剂治疗，观察组 (n=6) 在此基础上联合进行血浆置换治疗。比较 2 组患儿治疗前后的伯明翰血管炎评分(Birmingham vasculitis activity score，BVAS)评分、高分辨CT(high resolution CT，HRCT)评分、ANCA 转阴情况及肾功能恢复情况。结果2 组患儿治疗后的BVAS 评分(对照组：19.71±4.33 比11.57±2.64，t=4.247,P=0.001；观察组：20.50±5.85 比7.17±2.56，t= 3.552,P=0.005)及HRCT 评分(对照组：4.57±1.40 比2.57±1.51，t=2.384,P=0.034；观察组：4.17±1.17 比0.83±0.65, t=5.583,P＜0.001)均较治疗前有明显下降，而观察组BVAS 评分(7.17±2.56 比11.57±2.64，t=3.037,P=0.011)及HRCT 评分(0.83±0.65 比2.57± 1.51，t=2.413,P=0.034)的下降幅度明显大于对照组；观察组治疗后ANCA 转阴率明显高于对照组(P=0.007)；2 组患儿肾功能改善均不明显[对照组治疗前后肾小球滤过率：76.30(129.90,30.59)比98.88(141.89,43.10)ml/(min·1.73m2)，Z=0.943,P=0.346；观察组治疗前后肾小球滤过率：19.99(126.40,15.02)比17.80(145.19,12.45)ml/(min·1.73m2)，Z=0.689,P=0.491]。结论激素和免疫抑制剂治疗基础上联合进行血浆置换治疗儿童ANCA相关性血管炎能够更有效的清除ANCA，能更明显的降低患儿血管炎活动度及促进肺部病变恢复。

关键词: 抗中性粒细胞胞浆抗体相关性血管炎, 血浆置换, 儿童

Abstract: 【Abstract】Objective To observe the effect of plasma exchange on anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis in children. Methods A total of 13 patients with ANCA-associated vasculitis treated in Hunan Children's Hospital from January 2017 to January 2020 were enrolled in this study, They were divided into two groups: control group (n=7), treated with glucocorticoid and immunosuppressive therapy; observation group (n=6), treated with plasma exchange other than the treatment in control group. Birmingham vasculitis activity score (BVAS), high resolution CT (HRCT) score, rate of negative ANCA, and renal
function were compared between the two groups. Results After the treatment,BVAS and HRCTscore were significantly lower than those before treatment in the two groups (For BVAS: 19.71±4.33 vs. 11.57±2.64, t=4.247, P=0.001 in control group; 20.50 ± 5.85 vs. 7.17 ± 2.56, t=3.552, P=0.005 in observation group. For HRCT score: 4.57±1.40 vs. 2.57±1.51, t=2.384, P=0.034 in control group; 4.17±1.17 vs. 0.83±0.65, t=5.583, P<0.001 in observation group). The decreases of BVAS and HRCT score were more in observation group than in control group (For BVAS: 7.17 ± 2.56 vs. 11.57 ± 2.64, t=3.037, P=0.011; for HRCT score: 0.83 ± 0.65 vs. 2.57 ± 1.51, t=2.413, P=0.034). The rate of negative ANCA was significantly higher in observation group than in control group (P=0.007). No significant improvement of renal function was found in both groups [Glomerular filtration rate before and after the treatment: 76.30(129.90, 30.59) vs. 98.88(141.89, 43.10)ml/(min·1.73m2), Z=0.943, P=0.346 for control group; 19.99(126.40, 15.02) vs. 17.80(145.19, 12.45) ml/(min·1.73m2), Z=0.689, P=0.491 for observation group]. Conclusion Plasma exchange combined with glucocorticoid and immunosuppressive therapy, can efficiently clear ANCA, reduce the activity of vasculitis and promote the recovery of lung lesions in children with ANCA-associated vasculitis.

Key words: Anti-neutrophil cytoplasmic autoantibody-associated vasculitis, Plasma exchange, Child

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