Marinaki Smaragdi, Kompotiatis Panagiotis, Michelakis Ioannis, Stangou Maria, Papagianni Aikaterini, Koukoulaki Maria, Zerbala Synodi, Xydakis Dimitrios, Kaperonis Nikolaos, Dounousi Evangelia, Golfinopoulos Spyridon, Stefanidis Ioannis, Paikopoulou Aggeliki, Moustakas George, Stylianou Kostas, Tzanakis Ioannis, Papasotiriou Marios, Goumenos Dimitrios, Andrikos Aimilios, Kriki Pelagia, Panagoutsos Stylianos, Kiousi Eva, Grapsa Eirini, Koutroumpas Georgios, Pateinakis Panagiotis, Papadopoulou Dorothea, Liakopoulos Vasilios, Bacharaki Dimitra, Kouki Penelope, Petras Dimitrios, Bamichas Gerasimos, Boletis Ioannis
Department of Nephrology, Laiko General Hospital, National and Kapodistrian University, Athens, Greece.
Department of Nephrology, Hippokration General Hospital, Aristotle University, Thessaloniki, Greece.
PLoS One. 2024 Dec 18;19(12):e0315124. doi: 10.1371/journal.pone.0315124. eCollection 2024.
BACKGROUND/OBJECTIVE: Primary 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.
This 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.
We 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%), p<0.001), and higher percentage of relapses (135 (64%) vs. 27 (33%), p<0.001). Immunosuppression and its type (glucocorticoids vs. cyclosporine ± glucocorticoids) were not associated with renal survival.
In 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.
背景/目的：原发性局灶节段性肾小球硬化（FSGS）是特发性肾病综合征最常见的病因之一。我们的目的是描述一大群原发性FSGS患者，确定与较差肾脏存活率相关的危险因素，并评估不同免疫抑制方案对肾脏存活率的影响。
这是一项对1982年3月26日至2020年9月16日期间被诊断为原发性FSGS的成年人进行的历史性队列研究。主要结局是进展为终末期肾病（ESRD）。
我们纳入了579例患者。平均年龄为46（±15）岁，男性378例（65%），24小时蛋白尿中位数为3.8（2 - 6）g。在多变量分析中，只有估算肾小球滤过率（eGFR）（每增加1 ml/min，风险比（HR）：0.97，95%置信区间（CI）0.95 - 0.98）以及缓解状态（与未缓解相比，完全缓解（HR：0.03，95% CI 0.003 - 0.22）和部分缓解（HR：0.28，95% CI 0.13 - 0.61））与肾脏存活率相关。与未接受免疫抑制的患者相比，接受免疫抑制的患者中完全缓解的比例更高（121例（41%）对40例（24%），p<0.001），复发比例也更高（135例（64%）对27例（33%），p<0.001）。免疫抑制及其类型（糖皮质激素与环孢素±糖皮质激素）与肾脏存活率无关。
在原发性FSGS中，完全缓解和部分缓解与改善肾脏存活率相关。需要进一步的随机研究来评估不同治疗药物的疗效并指导治疗。
