Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, United Kingdom.
University of Manchester, Manchester, United Kingdom.
PLoS One. 2022 Oct 13;17(10):e0276053. doi: 10.1371/journal.pone.0276053. eCollection 2022.
Membranous nephropathy is the commonest cause of nephrotic syndrome in non-diabetic Caucasian adults over the age of 40 years. Primary membranous nephropathy is limited to the kidneys. Clinical management aims to induce remission, either spontaneously with supportive care, or with immunosuppression. Here, we describe the natural history of this condition in a large tertiary centre in the UK.
178 patients with primary membranous nephropathy were identified over 2 decades. We collected data on demographics, baseline laboratory values, treatment received and outcomes including progression to renal replacement therapy and death. Analysis was performed on the whole cohort and specific subgroups. Univariate and multivariate Cox regression was also performed.
Median age was 58.3 years with 63.5% male. Median baseline creatinine was 90μmol/L and urine protein-creatinine ratio 664g/mol. Remission (partial or complete) was achieved in 134 (75.3%), either spontaneous in 60 (33.7%) or after treatment with immunosuppression in 74 (41.6%), and of these 57 (42.5%) relapsed. Progression to renal replacement therapy was seen in 10.1% (much lower than classically reported) with mortality in 29.8%. Amongst the whole cohort, those who went into remission had improved outcomes compared to those who did not go into remission (less progression to renal replacement therapy [4.5% vs 28%] and death [20.1% vs 67%]. Those classified as high-risk (based on parameters including eGFR, proteinuria, serum albumin, PLA2R antibody level, rate of renal function decline) also had worse outcomes than those at low-risk (mortality seen in 52.6% vs 10.8%, p<0.001). The median follow-up period was 59.5 months.
We provide a comprehensive epidemiologic analysis of primary membranous nephropathy at a large tertiary UK centre. Only 10.1% progressed to renal replacement therapy. For novelty, the KDIGO risk classification was linked to outcomes, highlighting the utility of this classification system for identifying patients most likely to progress.
膜性肾病是 40 岁以上非糖尿病白种成年人肾病综合征最常见的病因。原发性膜性肾病仅局限于肾脏。临床治疗的目的是诱导缓解，要么是通过支持性治疗自发缓解，要么是通过免疫抑制治疗缓解。在这里，我们描述了在英国一家大型三级中心的这种疾病的自然病史。
在 20 多年的时间里，我们共发现了 178 名原发性膜性肾病患者。我们收集了人口统计学、基线实验室值、治疗方法和结果的数据，包括进展为肾脏替代治疗和死亡。对整个队列和特定亚组进行了分析。还进行了单变量和多变量 Cox 回归分析。
中位年龄为 58.3 岁，男性占 63.5%。中位基线肌酐为 90μmol/L，尿蛋白-肌酐比值为 664g/mol。134 名患者（75.3%）达到缓解（部分或完全），60 名患者（33.7%）自发缓解，74 名患者（41.6%）接受免疫抑制治疗后缓解，其中 57 名患者（42.5%）复发。10.1%的患者进展为肾脏替代治疗（远低于经典报道），死亡率为 29.8%。在整个队列中，与未缓解的患者相比，缓解的患者有更好的预后（进展为肾脏替代治疗的比例较低[4.5%对 28%]和死亡率[20.1%对 67%]）。根据 eGFR、蛋白尿、血清白蛋白、PLA2R 抗体水平、肾功能下降速度等参数分类为高危的患者（死亡率为 52.6%）比低危的患者（死亡率为 10.8%）预后更差（p<0.001）。中位随访时间为 59.5 个月。
我们在英国一家大型三级中心对原发性膜性肾病进行了全面的流行病学分析。只有 10.1%的患者进展为肾脏替代治疗。新颖之处在于，KDIGO 风险分类与预后相关，突出了该分类系统用于识别最有可能进展的患者的效用。
