UCL Centre for Nephrology, Royal Free Hospital, London, United Kingdom.
Clin J Am Soc Nephrol. 2013 Feb;8(2):219-24. doi: 10.2215/CJN.03680412. Epub 2012 Nov 15.
Induction therapy with oral cyclophosphamide (CYP) has been a mainstay of treatment in patients with severe renal failure secondary to ANCA-associated vasculitis (AAV). Recent evidence proposes using pulsed intravenous CYP in less severe disease to minimize adverse events. It is unclear if this can be translated to those with dialysis-dependent renal insufficiency.
DESIGN, SETTING, PARTICIPANTS, & METHODS: All AAV patients presenting between 2005 and 2010 requiring dialysis at presentation were retrospectively analyzed. Patients were treated with plasma exchange, corticosteroids, and intravenous CYP. Rate of dialysis independence at 3 and 12 months and adverse effects were assessed and compared with the outcome of the plasmapheresis, prednisolone, and oral CYP arm of the randomized MEPEX (methylprednisolone versus plasma exchange) trial.
Forty-one patients were included. At 3 months, 3 (7.3%) patients had died on dialysis, 12 (29.3%) remained dialysis dependent, and 26 (63.4%) were dialysis independent (creatinine, 2.5 mg/dl; GFR, 26 ml/min per 1.73 m(2)). Four patients subsequently reached ESRD at a median time of 83 days. Thirty-seven (90%) patients reached 1 year follow-up, 13 (35%) remained dialysis dependent, and 24 (65%) had independent renal function. Eleven patients (27%) had episodes of leukopenia (white cell count <4×10(9)/L) during CYP therapy and 17 (41%) experienced infectious complications. This compares favorably with the dialysis-dependent cohort treated with plasmapheresis in the MEPEX study in which 51% were alive with independent renal function at 1 year.
Intravenous CYP used with corticosteroids and plasmapheresis may be an effective alternative to oral CYP in patients with dialysis-dependent AAV.
对于因抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）导致的严重肾衰竭患者，口服环磷酰胺（CYP）诱导治疗一直是主要的治疗方法。最近的证据表明，在病情较轻的患者中使用脉冲式静脉注射 CYP 可以减少不良反应。但目前尚不清楚这种方法是否适用于那些依赖透析的肾功能不全患者。
回顾性分析了 2005 年至 2010 年间所有需要透析的 AAV 患者。患者接受了血浆置换、皮质类固醇和静脉注射 CYP 治疗。评估了 3 个月和 12 个月时的透析独立性率以及不良反应，并与随机 MEPEX（甲泼尼龙与血浆置换）试验中血浆置换、泼尼松龙和口服 CYP 组的结果进行了比较。
共纳入 41 例患者。在 3 个月时，3 例（7.3%）患者死于透析，12 例（29.3%）仍依赖透析，26 例（63.4%）实现了透析独立（肌酐 2.5mg/dl；GFR 26ml/min/1.73m2）。4 例患者随后在中位时间 83 天进入终末期肾病。37 例（90%）患者达到 1 年随访，13 例（35%）仍依赖透析，24 例（65%）有独立的肾功能。11 例（27%）患者在 CYP 治疗期间发生白细胞减少（白细胞计数<4×109/L），17 例（41%）发生感染并发症。与 MEPEX 研究中接受血浆置换治疗的依赖透析的患者相比，这一结果是有利的，在该研究中，1 年后 51%的患者有独立的肾功能且存活。
在依赖透析的 AAV 患者中，使用皮质类固醇和血浆置换的静脉注射 CYP 可能是口服 CYP 的有效替代方案。
