Ramachandran Raja, Prabakaran Rudreshwar, Priya Gnana, Nayak Saurabh, Kumar Pankaj, Kumar Ashwani, Kumar Vinod, Agrawal Neha, Rathi Manish, Kohli Harbir Singh, Nada Ritambhra
Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Nephron. 2022;146(2):138-145. doi: 10.1159/000518609. Epub 2021 Nov 3.
Renal dysfunction at presentation is uncommon in primary membranous nephropathy (PMN). The data on the outcome of PMN patients with renal dysfunction at outset are scarce. The objective of the current study was to report the clinical outcomes of PMN patients with renal dysfunction.
This prospective longitudinal observational study included PMN patients (both incident and treatment resistant) with an estimated glomerular filtration rate of <60 mL/min/1.73 m2. Immunosuppressive treatment was as per the unit's protocol. Patients were evaluated for proteinuria, creatinine, and serum albumin at monthly intervals for 6 months, then quarterly for a year, and then biannually. Both serum and tissue anti-PLA2R were performed at baseline.
Percentage of patients achieving clinical remission.
Sixty-four adults met study criteria and were analysed. The median (IQR) age of the patients was 48 (40, 56) years. PMN was PLA2R related in 52 (81.3%) patients. Twenty-eight (43.8%) and 30 (46.9%) patients were in remission at 12 months and at the end of the study [median (IQR) follow up: 24 months (12, 35)], respectively. Eight (12.5%) had progressed to end-stage renal disease at the last follow-up. Median (IQR) baseline anti-PLA2R titre was 150.1 RU/mL (38.5, 308). Nineteen (61.3%) and 18 (58.1%) patients with >90% reduction in anti-PLA2R titres at 12 months were in clinical remission at 12 months and at the end of the follow-up, respectively. Both cyclical cyclophosphamide/steroids (cCYC/GC) and rituximab were equally effective in inducing remission, but rituximab had a favourable adverse event profile compared to cCYC/GC.
To conclude, both cCYC/GC and rituximab are equally effective in inducing remission of nephrotic state with compromised renal function due to PMN. Immunosuppression induces remission in up to 50% PMN patients with CKD-stage 3-4.
初诊时出现肾功能不全在原发性膜性肾病（PMN）中并不常见。关于PMN患者一开始就存在肾功能不全时的预后数据很少。本研究的目的是报告患有肾功能不全的PMN患者的临床结局。
这项前瞻性纵向观察性研究纳入了估计肾小球滤过率<60 mL/min/1.73 m²的PMN患者（包括初发和治疗抵抗患者）。免疫抑制治疗按照科室方案进行。患者在6个月内每月评估蛋白尿、肌酐和血清白蛋白，然后在接下来的一年中每季度评估一次，之后每半年评估一次。在基线时检测血清和组织抗PLA2R。
达到临床缓解的患者百分比。
64名成年人符合研究标准并进行了分析。患者的中位（四分位间距）年龄为48（40，56）岁。52名（81.3%）患者的PMN与PLA2R相关。分别有28名（43.8%）和30名（46.9%）患者在研究结束时达到临床缓解，研究结束时的中位（四分位间距）随访时间为24个月（12，35）。8名（12.5%）患者在最后一次随访时进展为终末期肾病。基线抗PLA2R滴度的中位（四分位间距）值为150.1 RU/mL（38.5，308）。抗PLA2R滴度在12个月时降低>90%的患者中，分别有19名（61.3%）和18名（58.1%）在12个月时和随访结束时达到临床缓解。环磷酰胺/类固醇（cCYC/GC）和利妥昔单抗在诱导缓解方面同样有效，但与cCYC/GC相比，利妥昔单抗的不良事件谱更有利。
总之，cCYC/GC和利妥昔单抗在诱导因PMN导致肾功能受损的肾病综合征缓解方面同样有效。免疫抑制可使高达50%的CKD 3 - 4期PMN患者达到缓解。
