Nagappa M, Taly A B, Sinha S, Gayathri N, Bindu P S, Mahadevan A, Yasha T
Department of Neurology, NIMHANS, Bangalore, India.
J Clin Neuromuscul Dis. 2013 Jun;14(4):161-8. doi: 10.1097/CND.0b013e31828ee913.
To assess the therapeutic response of intravenous (IV) pulse cyclophosphamide therapy in polymyositis and dermatomyositis.
Data of 9 patients (M:F = 2:7) who received IV pulse cyclophosphamide therapy were retrospectively analyzed.
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.
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.
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。
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。
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。
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。