Mathrani Vinod, Alejmi Abdulfattah, Griffin Siân, Roberts Gareth
Department of Nephrology and Transplantation, University Hospital of Wales, Cardiff, UK.
Department of Nephrology, Ysbyty Gwynedd, Bangor, UK.
Clin Kidney J. 2017 Aug;10(4):450-454. doi: 10.1093/ckj/sfw152. Epub 2017 Mar 22.
Idiopathic membranous nephropathy (IMN) is one of the most common causes of nephrotic syndrome in adults. A proportion of patients will experience spontaneous remission and the decision to offer immunosuppression is guided by the presence of adverse prognostic features. Data relating to the efficacy of different immunosuppressive protocols is lacking, in particular there are little data available on the efficacy or benefits of an intravenous (IV) cyclophosphamide-based regimen. Since 2010, our unit has been using a treatment regimen based on IV cyclophosphamide and oral prednisolone for patients with IMN associated with adverse prognostic features. The outcomes of these patients were compared with a historic cohort of similar patients who did not receive immunosuppressive therapy.
Between January 2010 and 2014, a total of 41 patients were treated with pulse IV cyclophosphamide and oral prednisolone. The historical comparator group included 47 similar patients diagnosed between 2006 and 2010 who did not receive immunosuppression. Two-year follow-up data were collected. The primary outcome measure was time to remission of nephrotic syndrome (defined as normalization of serum albumin). Secondary outcomes included rate of progression of kidney disease as well as incidence of treatment-related adverse events.
As compared with supportive care alone, treatment with IV cyclophosphamide and oral prednisolone was associated with a significantly higher number of patients achieving remission. Within 18 months of therapy, 74% of treated patients had achieved a normal serum albumin level. Though there was a trend towards a more rapid decline in estimated glomerular filtration rate in the untreated cohort, this did not reach statistical significance. The IV cyclophosphamide-based regimen was well tolerated, with few significant treatment-associated side effects.
IV cyclophosphamide is a safe and effective treatment for IMN.
特发性膜性肾病(IMN)是成人肾病综合征最常见的病因之一。一部分患者会出现自发缓解,而是否给予免疫抑制治疗的决定取决于不良预后特征的存在。目前缺乏关于不同免疫抑制方案疗效的数据,尤其是关于基于静脉注射(IV)环磷酰胺方案的疗效或益处的数据很少。自2010年以来,我们科室一直使用基于IV环磷酰胺和口服泼尼松龙的治疗方案来治疗伴有不良预后特征的IMN患者。将这些患者的治疗结果与一组未接受免疫抑制治疗的类似患者的历史队列进行了比较。
在2010年1月至2014年期间,共有41例患者接受了静脉注射环磷酰胺冲击治疗和口服泼尼松龙治疗。历史对照组包括2006年至2010年期间诊断的47例未接受免疫抑制治疗的类似患者。收集了两年的随访数据。主要观察指标是肾病综合征缓解时间(定义为血清白蛋白正常化)。次要观察指标包括肾病进展率以及治疗相关不良事件的发生率。
与单纯支持治疗相比,静脉注射环磷酰胺和口服泼尼松龙治疗使达到缓解的患者数量显著增加。在治疗的18个月内,74%的治疗患者血清白蛋白水平恢复正常。尽管未治疗队列的估计肾小球滤过率有更快下降的趋势,但未达到统计学意义。基于IV环磷酰胺的方案耐受性良好,几乎没有显著的治疗相关副作用。
IV环磷酰胺是治疗IMN的一种安全有效的方法。