环磷酰胺每月静脉冲击联合糖皮质激素治疗难治性天疱疮14例临床分析  

Clinical Analysis of 14 Cases of Refractory Pemphigus Treated with Pulsed Intravenous Cyclophosphamide Monthly Combined with Corticosteroid

作　　者：李燕红[1] 张平[1] 周小勇[1] LI Yanhong;ZHANG Ping;ZHOU Xiaoyong(Department of Dematology,the First Hospital of Wuhan,Wuhan 430061,China)

机构地区：[1]武汉市第一医院皮肤科,湖北武汉430061

出　　处：《中国皮肤性病学杂志》2022年第3期300-303,共4页The Chinese Journal of Dermatovenereology

摘　　要：目的探讨环磷酰胺每月静脉冲击联合糖皮质激素治疗难治性天疱疮的临床疗效。方法收集本院2016年1月-2019年6月,经常规治疗无效或有多种并发症而采用大剂量环磷酰胺(1 g/次,1次/月)联合糖皮质激素[1~1.5 mg/(kg·d)]治疗的14例天疱疮住院患者的临床资料,并对其临床治疗情况及疗效进行回顾性分析。结果14例经治疗的患者中,皮疹痊愈8例,显效1例,好转2例,无效3例。患者中死亡2例。所有患者的治疗次数为1~9次。皮损控制时间为2~31 d,平均13.3 d,皮损痊愈时间为29~120 d,平均79.9 d。9例患者1个月后激素减量相当于泼尼松10~30 mg/d,4例达到临床痊愈的患者激素减量至10~20 mg/d,糖皮质激素减量最大幅度为80%。最常见的并发症是感染。结论环磷酰胺每月静脉冲击联合糖皮质激素治疗天疱疮能有效控制皮疹,减少激素减量过程中病情复发,适用于大面积,经常规治疗无效的重症天疱疮。但患者选择需慎重,使用期间需监测不良反应。Objective To investigate the clinical efficacy of pulsed intravenous cyclophosphamide monthly combined with corticosteroid in the treatment of 14 patients with refractory pemphigus.Methods The clinical data were collected from 14 hospitalized pemphigus patients who received high-dose cyclophosphamide(1 g per time,once a month)combined with corticosteroid[1-1.5 mg/(kg·d)]from January 2016 to June 2019 due to ineffective conventional treatment or multiple complications in our hospital.The clinical treatment and curative effect were analyzed retrospectively.Results Of the 14 treated patients,eight patients achieved complete clinical remission,one had an excellent response,two had a good response,three patients had no clinical response,and two died.The number of treatment for these patients varied 1 to 9.The time for skin control was 2-31 d,with an average of 13.3 d.The time for skin lesions to heal was 29-120 d,with an average of 79.9 d.Of them,9 patients reduced corticosteroid dose equivalent to 10-30 mg/d of prednisone after one month.The corticosteroid dose of 4 patients who achieved complete clinical remission were reduced to 10 to 20 mg/d,with a maximum reduction of 80%.The most common complication was infection.Conclusion Pulsed intravenous cyclophosphamide monthly combined with corticosteroid can effectively control the rash and reduce the recurrence of pemphigus in the process of corticosteroid reduction.It is appropriate for the treatment of pemphigus with large rash area and severe pemphigus with poor response to conventional therapy.I its adverse effects should be considered prior to careful patient selection and closely monitored in patients on treatment.

关 键 词：天疱疮 冲击治疗 环磷酰胺 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]