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。
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.
作者
钮含春
王晓军
戈海青
谷文佳
王春艳
霍泉金
NIU Han-chun;WANG Xiao-jun;GE Hai-qing;GU Wen-jia;WANG Chun-yan;HUO Quan-jin(Department of Rheumatology,Ping’an Hospital,Hebei Medical University,Shijiazhuang 050021,China)
出处
《中国血液净化》
CSCD
2020年第5期314-316,356,共4页
Chinese Journal of Blood Purification
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
Melanoma differentiation-associated gene 5
Dermatomyositis
Rapidly progressive interstitial lung disease
Hemoperfusion
Treatment
