Sandoval Diego, Poveda Rafael, Draibe Juliana, Pérez-Oller Laureà, Díaz Montserrat, Ballarín José, Saurina Anna, Marco Helena, Bonet Josep, Barros Xoana, Fulladosa Xavier, Torras Joan, Cruzado Josep M
Department of Nephrology, Hospital de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain.
Department of Nephrology, Hospital General de Vic, Vic, Catalunya, Spain.
Clin Kidney J. 2017 Oct;10(5):632-638. doi: 10.1093/ckj/sfx035. Epub 2017 Apr 27.
This study assessed the efficacy of therapy with mycophenolate (MF) and reduced doses of steroids in adults with steroid-dependent/frequently relapsing idiopathic nephrotic syndrome (SD/FR-INS).
Twenty-nine nephrotic patients (including 16 males and 13 females; mean age: 40 years, range: 18-74) were treated. Starting doses of MF were 2000 mg/day for mofetil MF (1500 mg/day in one patient) or 1440 mg/day for sodium MF. The initial prednisone (PDN) dose was 10 mg/day in 14 patients, 5 mg/day in two patients and no steroids in one patient. In the remaining 12 patients, moderate initial doses of PDN were administered (mean: 23.7 mg/day, range: 15-40), tapering to 10 mg/day after 1 month.
Nephrotic syndrome remission was achieved in 27/29 cases (93.1%) (25 complete, 2 partial). Two patients showed resistance to the prescribed schedule. The first cycle of MF therapy was concluded in 20 patients after a mean (range) of 16.9 months (12-49). Maintenance of remission was observed in 11 of these 20 cases (55%) after a mean follow-up of 32.8 months (12-108). In nine patients with nephrotic syndrome relapse after tapering of MF (MF dependency), the same MF-PDN schedule was restarted, leading again to remission in all nine. The remaining seven MF-sensitive patients are still receiving their first therapeutic cycle. To date, the mean time under therapy in the 27 MF-sensitive patients is 38 months (4-216). Regarding complications, only minor digestive disorders and a slight decrease in blood haemoglobin levels were observed in a few patients.
MF plus reduced doses of PDN is an effective and well-tolerated therapy for adult SD/FR-INS. Though MF dependence is observed, its low toxicity could allow long periods of therapy if it is required to maintain nephrotic syndrome remission.
本研究评估了霉酚酸酯（MF）联合减量糖皮质激素治疗成人激素依赖型/频繁复发的特发性肾病综合征（SD/FR-INS）的疗效。
对29例肾病患者（16例男性，13例女性；平均年龄40岁，范围18 - 74岁）进行治疗。霉酚酸酯（MMF）起始剂量为2000mg/天（1例患者为1500mg/天），霉酚酸钠起始剂量为1440mg/天。14例患者初始泼尼松（PDN）剂量为10mg/天，2例患者为5mg/天，1例患者未使用糖皮质激素。其余12例患者初始给予中等剂量的PDN（平均：23.7mg/天，范围15 - 40mg/天），1个月后逐渐减至10mg/天。
29例患者中27例（93.1%）实现肾病综合征缓解（25例完全缓解，2例部分缓解）。2例患者对既定治疗方案耐药。20例患者在平均（范围）16.9个月（12 - 49个月）后完成了MF治疗的第一个周期。这20例患者中11例（55%）在平均随访32.8个月（12 - 108个月）后维持缓解。9例在MF减量后肾病综合征复发（MF依赖）的患者重新开始相同的MF - PDN治疗方案，所有9例再次缓解。其余7例对MF敏感的患者仍在接受第一个治疗周期。迄今为止，27例对MF敏感的患者平均治疗时间为38个月（4 - 216个月）。关于并发症，仅在少数患者中观察到轻微的消化系统紊乱和血红蛋白水平略有下降。
MF联合减量的PDN是治疗成人SD/FR-INS的一种有效且耐受性良好的疗法。尽管观察到MF依赖，但如果需要维持肾病综合征缓解，其低毒性允许进行长时间治疗。
