中国血液净化

中国血液净化 ›› 2020, Vol. 19 ›› Issue (05): 314-316.doi: 10.3969/j.issn.1671-4091.2020.05.007

• 临床研究 • 上一篇    下一篇

血液灌流联合药物治疗抗黑色素瘤分化相关基因-5 抗体阳性皮肌炎合并快速进展性间质性肺病12 例回顾性分析

钮含春1,王晓军1,戈海青1,谷文佳1,王春艳1,霍泉金1   

  1. 1.河北医科大学附属石家庄平安医院风湿免疫科
  • 收稿日期:2019-12-23 修回日期:2020-03-18 出版日期:2020-05-12 发布日期:2020-05-12
  • 通讯作者: 王晓军 wangxiaojun0826@163.com E-mail:wangxiaojun0826@163.com

Treatment of anti- MDA5 positive dermatomyositis complicated with rapidly progressive interstitial lung disease by hemoperfusion and drugs: a retrospective analysis of 12 cases 

  1.  1Department of Rheumatology,Ping’an Hospital, Hebei Medical University, Shijiazhuang 050021, China
  • Received:2019-12-23 Revised:2020-03-18 Online:2020-05-12 Published:2020-05-12

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摘要： 【摘要】目的探讨血液灌流联合甲泼尼龙、他克莫司和/或环磷酰胺等治疗抗黑色素瘤分化相关基因-5(melanoma differentiation-associated gene 5,MDA5)抗体阳性皮肌炎合并快速进展性间质性肺病的疗效和安全性。方法回顾性分析2016 年3 月～2018 年11 月在河北医科大学附属石家庄平安医院治疗的抗MDA5 阳性皮肌炎合并快速进展性间质性肺病12 例患者，均使用HA280 树脂灌流器进行血液灌流治疗，同时联合应用甲泼尼龙、他克莫司和/或环磷酰胺等药物。收集患者治疗前后外周血淋巴细胞计数、白介素(interleukin, IL)-6、IL-10、C 反应蛋白、肺泡Ⅱ型细胞表面抗原-6(Krebs Von den Lungen-6,KL-6)、铁蛋白、动脉血氧分压、动脉血二氧化碳分压、肺部高分辨CT 评分等指标。治疗前、治疗后2 周及4 周间比较采用方差分析，两两比较采用LSD-t 检验。结果2 例死亡，其余10 例患者随访至今存活。经过4周治疗与治疗前对比，血清IL-6、IL-10、KL-6、铁蛋白、C 反应蛋白明显下降(F 值分别为6.593, 9.029, 7.872, 8.760, 20.490；P 值分别为0.005，0.001，0.002，0.001，＜0.001)，外周血淋巴细胞计数、动脉血氧分压、动脉血二氧化碳分压明显改善(F 值分别为16.810,3.580,3.467；P 值分别为＜0.001，0.042，0.046)，肺部高分辨CT 评分改善(F=2.073, P=0.145)，但无统计学意义。结论血液灌流联合甲泼尼龙、他克莫司和/或环磷酰胺等治疗抗MDA5 抗体阳性皮肌炎合并快速进展性间质性肺病具有较好疗效，且安全性较好。

关键词: 抗黑色素瘤分化相关基因-5, 皮肌炎, 快速进展性间质性肺病, 血液灌流, 治疗

Abstract: 【Abstract】Objective To investigate the efficacy and safety of hemoperfusion combined with drugs for the treatment of anti-MDA5 positive dermatomyositis complicated with rapidly progressive interstitial lung disease (RP- ILD). Methods We retrospectively studied 12 anti-MDA5 positive dermatomyositis patients complicated with RP-ILD hospitalized in the period from March 2016 to November 2018. They were treated with HA-280 resin hemoperfusion apparatus for hemoperfusion and the drugs of methylprednisolone, tacrolimus and/or cyclophosphamide. Their peripheral blood lymphocyte count, serum IL-6, IL-10, CRP, Krebs Von den Lungen-6 (KL-6) and ferritin, arterial partial pressure of O2 and CO2, and lung high resolution computed
tomography (HRCT) score were recruited before and after the treatment. ANOVA was used to analyze the data before the treatment and after the treatment for 2 and 4 weeks. LSD-t test was used for the comparison of two groups of data. Results After the treatment for 4 weeks, serum IL-6、IL-10 、KL-6 、ferritin and CRP decreased significantly (F=6.593, 9.029, 7.872, 8.760, 20.490；P=0.005, 0.001, 0.002, 0.001, ＜0.001). Arterial O2 partial pressure、arterial CO2 partial pressure、peripheral blood lymphocyte count improved significantly (F=3.580, 3.467, 16.810；P=0.042,0.046,＜0.001), as compared to those before the treatment. HRCT score (F=2.073, P=0.145) also improved but without statistical significance. Conclusion Hemoperfusion combined with methylprednisolone, tacrolimus and/or cyclophosphamide were effective and safe for the treatment of anti-MDA5 positive dermatomyositis complicated with RP-ILD.

Key words: Melanoma differentiation-associated gene 5, Dermatomyositis, Rapidly progressive interstitial lung disease, Hemoperfusion, Treatment

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