Deleersnijder Dries, Cleenders Evert, Coemans Maarten, Dendooven Amélie, Koshy Priyanka, Claes Kathleen, De Vusser Katrien, Meijers Björn K, Sprangers Ben, Van Laecke Steven, Van Craenenbroeck Amaryllis H
Nephrology and Renal Transplantation Research Group, Department of Microbiology, Immunology and Transplantation, KU Leuven, Leuven, Belgium.
Division of Pathology, University Hospital Ghent, Ghent, Belgium.
Clin Kidney J. 2025 Feb 21;18(4):sfaf060. doi: 10.1093/ckj/sfaf060. eCollection 2025 Apr.
Studies that compare kidney outcomes across patients with different forms of focal segmental glomerulosclerosis (FSGS) are lacking.
This retrospective study compared annual estimated glomerular filtration rate (eGFR) slope and kidney failure rate (eGFR <15 mL/min/1.73 m or initiation of kidney replacement therapy) across patients with biopsy-proven primary, maladaptive, genetic and undetermined FSGS. Patients were included from two Belgian tertiary referral hospitals, from 2010 until 2022. Associations between covariates and kidney failure were estimated using Cox and Fine and Gray models. eGFR slopes were estimated using linear mixed-effects models.
Eighty-two patients were subdivided into primary (28.1%), maladaptive (40.2%), genetic (14.6%) and undetermined FSGS (17.1%) groups. Kidney failure occurred in 22 patients (26.8%). Primary FSGS patients exhibited higher baseline eGFR and less chronic changes on biopsy. The annual eGFR slope was -2.5 mL/min in primary, -2.5 mL/min in maladaptive, -4.6 mL/min in genetic and -4.4 mL/min in undetermined FSGS. Female sex was associated with a lower kidney failure rate and higher eGFR slope. Higher proteinuria at biopsy was associated with a higher kidney failure rate, lower eGFR slope and a higher mortality rate. Global sclerosis on kidney biopsy was associated with lower baseline eGFR, while a higher percentage of segmental sclerosis rather associated with more rapid eGFR decline [-1.5 mL/min/year per 10% increase, 95% confidence interval (-2.2, -0.7)].
Patients with primary FSGS were biopsied earlier in their disease course and exhibited surprisingly good kidney outcome. Overall, sex, baseline eGFR, proteinuria and the degree of focal and global glomerulosclerosis play a more important role in estimating the prognosis of patients with FSGS than merely the FSGS etiology.
缺乏对不同形式局灶节段性肾小球硬化（FSGS）患者的肾脏结局进行比较的研究。
这项回顾性研究比较了经活检证实的原发性、适应性不良性、遗传性和未定型FSGS患者的年度估计肾小球滤过率（eGFR）斜率和肾衰竭发生率（eGFR<15 mL/min/1.73 m²或开始肾脏替代治疗）。研究纳入了2010年至2022年期间比利时两家三级转诊医院的患者。使用Cox模型以及Fine和Gray模型估计协变量与肾衰竭之间的关联。使用线性混合效应模型估计eGFR斜率。
82例患者被分为原发性（28.1%）、适应性不良性（40.2%）、遗传性（14.6%）和未定型FSGS（17.1%）组。22例患者（26.8%）发生了肾衰竭。原发性FSGS患者的基线eGFR较高，活检时的慢性改变较少。原发性FSGS患者的年度eGFR斜率为-2.5 mL/min，适应性不良性为-2.5 mL/min，遗传性为-4.6 mL/min，未定型FSGS为-4.4 mL/min。女性与较低的肾衰竭发生率和较高的eGFR斜率相关。活检时较高的蛋白尿与较高的肾衰竭发生率、较低的eGFR斜率和较高的死亡率相关。肾脏活检时的全球硬化与较低的基线eGFR相关，而节段性硬化比例较高则与eGFR下降更快相关[-每增加10%，下降-1.5 mL/min/年，95%置信区间（-2.2，-0.7）]。
原发性FSGS患者在病程中更早接受活检，且肾脏结局出人意料地良好。总体而言，性别、基线eGFR、蛋白尿以及局灶性和全球性肾小球硬化程度在评估FSGS患者预后方面比单纯的FSGS病因发挥着更重要的作用。
