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脉冲环磷酰胺疗法治疗多发性肌炎和皮肌炎的疗效及局限性

Efficacy and limitations of pulse cyclophosphamide therapy in polymyositis and dermatomyositis.

作者信息

Nagappa M, Taly A B, Sinha S, Gayathri N, Bindu P S, Mahadevan A, Yasha T

机构信息

Department of Neurology, NIMHANS, Bangalore, India.

出版信息

J Clin Neuromuscul Dis. 2013 Jun;14(4):161-8. doi: 10.1097/CND.0b013e31828ee913.

Abstract

OBJECTIVES

To assess the therapeutic response of intravenous (IV) pulse cyclophosphamide therapy in polymyositis and dermatomyositis.

METHODS

Data of 9 patients (M:F = 2:7) who received IV pulse cyclophosphamide therapy were retrospectively analyzed.

RESULTS

The mean symptom duration was 11.33 ± 10.6 months (range, 2-34 months). The cohort comprised (1) primary idiopathic polymyositis (n = 1), (2) primary idiopathic dermatomyositis (n = 1), (3) childhood type associated with vasculitis (n = 1), and (4) associated with collagen vascular disease (n = 6). All patients improved and became clinically asymptomatic after a mean period of 12.33 ± 6.5 months (range, 4-24 months); 5 remained asymptomatic at the end of a median follow-up period of 22 months. All patients received concomitant steroid therapy, and in 6, steroids could be tapered after the initiation of IV pulse cyclophosphamide therapy.

CONCLUSIONS

In this cohort of polymyositis/dermatomyositis, treatment with IV pulse cyclophosphamide was associated with improvement; the therapeutic response was sustained in majority of the patients.

摘要

目的

评估静脉注射脉冲环磷酰胺疗法对多发性肌炎和皮肌炎的治疗反应。

方法

回顾性分析9例接受静脉注射脉冲环磷酰胺疗法患者(男:女 = 2:7)的数据。

结果

平均症状持续时间为11.33±10.6个月(范围2 - 34个月)。该队列包括:(1)原发性特发性多发性肌炎(n = 1),(2)原发性特发性皮肌炎(n = 1),(3)与血管炎相关的儿童型(n = 1),以及(4)与胶原血管病相关(n = 6)。所有患者在平均12.33±6.5个月(范围4 - 24个月)后病情改善并临床无症状;在中位随访期22个月结束时,5例仍无症状。所有患者均接受了联合类固醇治疗,其中6例在开始静脉注射脉冲环磷酰胺治疗后可逐渐减少类固醇用量。

结论

在该多发性肌炎/皮肌炎队列中,静脉注射脉冲环磷酰胺治疗带来病情改善;大多数患者的治疗反应得以持续。

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