血浆置换治疗抗MDA5阳性皮肌炎伴肺间质纤维化临床疗效及安全性分析

doi:10.20223/j.cnki.1000-8535.2024.10.011

摘要/Abstract

摘要： 目的探讨血浆置换联合泼尼松（PDN）联合环磷酰胺（CTX）治疗抗黑色素瘤分化相关基因5（MDA5）抗体阳性皮肌炎（DM）伴肺间质纤维化（ILD）患者疗效及安全性。方法回顾性分析2014年6月—2023年6月普洱市人民医院诊断的MDA5阳性DM伴ILD患者40例,其中治疗组20例,采用血浆置换联合PDN 1 mg/kg每日1次口服,4周后减量,每周减总量10%,CTX 1 g每月1次静脉滴注,共6次治疗;另20例设为对照组,采用PDN 1 mg/kg每日1次口服,4周后减量,每周减总量10%,CTX 1 g每月1次静脉滴注,共6次治疗,分别于治疗后3月、6月检测一氧化碳弥散量（DLCO）、第1秒用力呼气量（FEV₁）,血清铁蛋白（SF）、C-反应蛋白（CRP）、涎液化糖链抗原（KL-6）、MDA5转阴率行疗效评估。结果在治疗3个月和6个月时,两组患者的DLCO、FEV1、SF、CRP、KL-6、MDA5转阴率等指标的完全缓解率不一致。其中,3个月时,治疗组上述指标的完全缓解率依次为95%、85%、90%、90%、90%、85%,对照组依次为15%、20%、20%、15%、0%、0%。两组患者在治疗3个月的DLCO、FEV1、SF、CRP、KL-6水平和MDA5转阴数均有所不同。其中治疗组的DLCO、KL-6、CRP水平均较对照组降低（P<0.01）,治疗组FEV₁水平较对照组升高（P<0.01）,治疗组SF水平较对照组降低（P<0.05）,两组治疗6个月时,治疗组上述指标缓解率依次为95%、85%、90%、90%、90%、85%,对照组依次为20%、25%、20%、20%、20%、5%。两组患者在DLCO、FEV₁、SF、CRP、KL-6水平以及MDA5转阴数和死亡例数方面比较差异均有统计学意义,其中治疗组的DLCO、KL-6和CRP水平均较对照组降低（P<0.01）,治疗组FEV₁水平较对照组升高（P<0.01）,SF水平治疗组较对照组降低（P<0.05）。结论在MDA5抗体阳性DM伴ILD患者治疗中,给予血浆置换联合PDN、CTX治疗,可以提高疗效,降低病死率。

关键词: 抗MDA5抗体阳性皮肌炎, 环磷酰胺, 血浆置换, 肺间质纤维化

Abstract: Objective To explore the effect of plasmapheresis combined with prednisone（PDN）plus cytoxan（CTX）on patients with anti-melanoma differentiation-associated gene 5（MDA 5）antibody-positive dermatomyositis（DM）with interstitial lung disease（ILD）. Methods The data of 40 patients with MDA 5 positive DM and ILD diagnosed in the People's Hospital of Pu'er City from June 2014 to June 2023 were retrospectively was analyzed．Twenty patients of the treatment group were treated with plasmapheresis combined with PDN 1mg / kg once daily,which was reduced by 10% per week after 4 weeks．The other 20 patients of the control group were treated with PDN 1mg / kg once daily,which was reduced after 4 weeks by 10% per week,and CTX 1g once per month．diffusing capacity of the lungs for carbon monoxide（DLCO）,forced expiratory volume in the first second（FEV₁）,serum ferritin（SF）,C-reactive protein（CRP）,Krebs Von den Lungen-6（KL-6）and MDA5 negative conversion rate were measured at 3 and 6 months after treatment,respectively. Results At 3 and 6 months of treatment,complete remission rates of DLCO,FEV₁,SF,CRP,KL-6,MDA 5 conversion and other indicators were inconsistent．Among them,at 3 months,the complete response rate of the above indicators in the treatment group was successively：95%,85%,90%,90%,90% and 85%．The control group was 15%,20%,20%,15%,and 0%,0%．Statistical analysis showed that the levels of DLCO,FEV₁,SF,CRP,KL-6 and MDA 5 significantly varied at 3 months of treatment．Pairwise comparison of LSD found that the DLCO,KL-6 and CRP levels in the treatment group were significantly lower than the control group（P<0.01）,the FEV₁ level in the treatment group was significantly higher（P<0.01）,and the SF level in the treatment group was significantly lower（P<0.05）．After 6 month of treatment,the complete response rate of the above indicators in the treatment group were 95%,85%,90%,90%,90% and 85%,and the complete response rate of the above indicators in the control group was 20%,25%,20%,20%,20% and 5%．Statistical analysis showed the levels of DLCO,FEV₁,SF,CRP,KL-6 for the amount of MDA 5 and the number of deaths between two groups were significantly different．Further pairwise comparison of LSD showed that the DLCO,KL-6 and CRP levels in the treatment group were significantly lower compared with the control group（P<0.01）,the FEV₁ level was significantly increased compared with the control group（P<0.01）,and the SF treatment group was significantly decreased compared with the control group（P<0.05）. Conclusions In the treatment of patients with MDA 5 antibody positive DM with ILD,the treatment of plasmapheresis combined with PDN and CTX can significantly improve the efficacy and reduce the mortality rate．

Key words: anti-melanoma differentiation-associated gene 5（MDA5）dermatomyositis, cytoxan, plasmapheresis, interstitial lung disease

郑朝恩, 郑迪, 刘正海, 罗娇, 田应波, 刘丽. 血浆置换治疗抗MDA5阳性皮肌炎伴肺间质纤维化临床疗效及安全性分析[J]. 广州医药, 2024, 55(10): 1166-1171.

ZHENG Chao'en, ZHENG Di, LIU Zhenghai, LUO Jiao, TIAN Yingbo, LIU Li. Clinical efficacy and safety analysis of plasmapheresis for anti-MDA 5 positive dermatomositis with lung interstitial fibrosis[J]. Guangzhou Medical Journal, 2024, 55(10): 1166-1171.

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