Baseline characteristics of the cohort.

Background/ObjectivePrimary Focal and Segmental glomerulosclerosis (FSGS) is one of the most common causes of idiopathic nephrotic syndrome. Our aim was to describe a large cohort of patients with primary FSGS, identify risk factors associated with worse renal survival and assess the impact of different immunosuppressive regiments on renal survival.MethodsThis was a historical cohort study of adults who were diagnosed with primary FSGS from March 26, 1982, to September 16, 2020. The primary outcome was progression to ESRD.ResultsWe included 579 patients. The mean age was 46 (±15) years of age, with 378 (65%) males and median 24-hour proteinuria was 3.8 (2–6) g. In multivariable analysis only eGFR (HR: 0.97 per ml/min increase, 95% CIs 0.95–0.98) and remission status (complete remission (HR: 0.03, 95% CIs 0.003–0.22) and partial remission (HR: 0.28, 95% CIs 0.13–0.61) compared to no remission) were associated with renal survival. Among patients who received immunosuppression compared to those that did not, there was a higher percentage of complete remission (121 (41%) vs. 40 (24%), pConclusionIn primary FSGS, complete and partial remission were associated with improved renal survival. Further randomized studies are needed to assess the efficacy of different therapeutic agents and guide treatment.研究背景与目的 原发性局灶节段性肾小球硬化症（focal segmental glomerulosclerosis, FSGS）是特发性肾病综合征最常见的致病原因之一。本研究旨在描述一组大样本原发性FSGS患者队列，明确与不良肾脏预后相关的危险因素，并评估不同免疫抑制治疗方案对肾脏存活率的影响。 方法 本研究为一项回顾性队列研究，纳入1982年3月26日至2020年9月16日期间确诊为原发性FSGS的成年患者。本研究的主要结局为进展至终末期肾病（end-stage renal disease, ESRD）。 结果 本研究共纳入579例患者。患者平均年龄为46（±15）岁，其中男性378例，占比65%，中位24小时尿蛋白定量为3.8（2~6）g。多变量分析结果显示，仅估算肾小球滤过率（estimated glomerular filtration rate, eGFR）（风险比：每ml/min升高对应HR=0.97，95%置信区间0.95~0.98）与缓解状态（相较于未缓解，完全缓解（HR=0.03，95%置信区间0.003~0.22）与部分缓解（HR=0.28，95%置信区间0.13~0.61））与肾脏存活率显著相关。与未接受免疫抑制治疗的患者相比，接受免疫抑制治疗的患者完全缓解率更高（分别为121例[41%]与40例[24%]，P<0.05）。 结论 在原发性FSGS患者中，完全缓解与部分缓解均与肾脏存活率改善密切相关。未来仍需开展更多随机对照研究，以评估不同治疗药物的疗效并指导临床诊疗。