K. Tselios, MD, PhD, D.D. Gladman, MD, FRCPC, C. Taheri, BHSc, J. Su, MB, MSc, M.B. Urowitz, MD, FRCPC, University of Toronto Lupus Clinic, Centre of Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, Ontario, Canada.
J Rheumatol. 2021 Feb;48(2):228-231. doi: 10.3899/jrheum.200161. Epub 2020 Sep 1.
Lupus nephritis (LN) may lead to endstage kidney disease (ESKD) in 22% of patients over a period of 15 years, with the risk being particularly higher in diffuse proliferative forms. The rate of kidney function decline varies. However, a catastrophic course leading to ESKD within a few years from onset is uncommon. The aim of the present study was to assess the factors associated with rapid progression to ESKD in patients with LN.
Patients from the Toronto Lupus Clinic with biopsy-proven LN at presentation and estimated glomerular filtration rate (eGFR) 60 mL/min/1.73 m, who developed ESKD within 3 years were retrieved. Pathology reports were reviewed with particular emphasis on distinct histopathologic features. Demographic, clinical, laboratory, and therapeutic variables were also analyzed.
Ten patients (1.8% of the total LN population) developed ESKD within 3 years of diagnosis. Their mean age was 34.2 ± 7.3 years, mean time to ESKD 19.2 ± 12.4 months, initial eGFR 90.2 ± 24.9 mL/min/1.73 m, proteinuria 2.7 ± 1.04 g/24 h. The median rate of kidney function decline was > 43 mL/min/1.73 m/year. One patient had LN class III, 5 had LN class IV, 2 had membranous LN (class V), and another 2 had mixed IV/V. Moreover, 2 patients had extensive thrombotic microangiopathy, 1 collapsing glomerulonephritis, and 1 concomitant antiglomerular basement membrane (anti-GBM) nephropathy. Four patients showed no unusual kidney pathology; all of them had severe noncompliance (discontinued all medications to follow alternative treatment).
Catastrophic progression to ESKD is uncommon in LN. The major associated factors are poor compliance and distinct histopathologic features such as thrombotic microangiopathy, collapsing glomerulopathy, and concomitant anti-GBM nephropathy.
狼疮肾炎（LN）可导致 22%的患者在 15 年内发展为终末期肾病（ESKD），弥漫性增生性病变的风险尤其高。肾功能下降的速度各有不同。然而，从发病到 ESKD 仅在数年内迅速进展的灾难性病程并不常见。本研究旨在评估 LN 患者快速进展为 ESKD 的相关因素。
从多伦多狼疮诊所检索了经活检证实的 LN 患者的资料，这些患者在发病时肾小球滤过率（eGFR）为 60mL/min/1.73m，在 3 年内发展为 ESKD。特别强调了不同的组织病理学特征，对病理报告进行了审查。还分析了人口统计学、临床、实验室和治疗变量。
10 名患者（LN 总人群的 1.8%）在诊断后 3 年内发展为 ESKD。他们的平均年龄为 34.2 ± 7.3 岁，ESKD 发病时间为 19.2 ± 12.4 个月，初始 eGFR 为 90.2 ± 24.9mL/min/1.73m，蛋白尿为 2.7 ± 1.04g/24h。肾功能下降的中位速度大于 43mL/min/1.73m/年。1 例为 LN Ⅲ级，5 例为 LN Ⅳ级，2 例为膜性 LN（V 级），另 2 例为混合性 IV/V 级。此外，2 例患者有广泛的血栓性微血管病，1 例有塌陷性肾小球肾炎，1 例有抗肾小球基底膜（anti-GBM）肾病。4 例患者的肾脏病理无异常；他们都有严重的不遵医行为（停止所有药物治疗，转而接受替代治疗）。
LN 患者迅速进展为 ESKD 并不常见。主要的相关因素是不遵医嘱和特定的组织病理学特征，如血栓性微血管病、塌陷性肾小球病和同时存在的抗 GBM 肾病。
