Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy.
Unit of Radiology, ASST Papa Giovanni XXIII, Bergamo, Italy.
Nephron. 2024;148(6):367-378. doi: 10.1159/000534893. Epub 2023 Nov 3.
Primary membranoproliferative glomerulonephritis (MPGN) is a rare kidney disease with poor prognosis and no specific therapies. The disease heterogeneity and the difficulty of performing repeated kidney biopsies pose big challenges. This study investigates the correlation between non-contrast enhanced magnetic resonance imaging (MRI) and histologic and clinical findings in patients with primary MPGN.
Patients with primary MPGN underwent baseline and 1-year kidney MRI in addition to biopsy and laboratory testing as part of a prospective MRI subproject of a clinical trial (
Seven patients with primary MPGN (18[17-21] years, 43% females) were included. Kidney biopsies showed variable degree of global and segmental glomerular sclerosis ([5-30]% and [10-60]%), mild interstitial fibrosis (<10%), and increased peritubular interstitial volume ([19-40]%). MRI and laboratory parameters changed very differently from patient to patient over 1 year. Peritubular interstitial volume and glomerular sclerosis negatively associated with renal blood flow (RBF) (rho = -0.81 and -0.77), and positively with renal vascular resistance (RVR) (rho = 0.65 and 0.73). Urinary albumin to creatinine ratio (uACR) negatively associated with RBF and filtration fraction (FF) (rho = -0.86 and -0.6), while positively with RVR (rho = 0.88). uACR decrease was associated with kidney diffusivity increase (rho = -0.5). Measured glomerular filtration rate (GFR) positively associated with kidney diffusivity, RBF, and FF (rho = 0.87, 0.85, and 0.59), while negatively with RVR (rho = -0.89); GFR increase was associated with kidney diffusivity, RBF, and FF increase (rho = 0.77, 0.7, and 0.7) and RVR decrease (rho = -0.7).
The strong correlation found between MRI and histologic and clinical findings, despite the rather limited number of patients, highlights MRI potential to monitor disease progression in patients with rare kidney disease.
原发性膜增生性肾小球肾炎(MPGN)是一种预后不良且无特异性治疗方法的罕见肾脏疾病。疾病异质性和重复进行肾活检的难度带来了巨大挑战。本研究旨在探讨原发性 MPGN 患者的非对比增强磁共振成像(MRI)与组织学和临床发现之间的相关性。
作为一项临床试验(
本研究纳入了 7 名患有原发性 MPGN 的患者(18[17-21]岁,43%为女性)。肾脏活检显示不同程度的肾小球整体和节段性硬化([5-30]%和[10-60]%)、轻度间质纤维化(<10%)和肾小管周围间质体积增加([19-40]%)。1 年内,每位患者的 MRI 和实验室参数变化非常不同。肾小管周围间质体积和肾小球硬化与肾血流量(RBF)呈负相关(rho = -0.81 和 -0.77),与肾血管阻力(RVR)呈正相关(rho = 0.65 和 0.73)。尿白蛋白与肌酐比值(uACR)与 RBF 和滤过分数(FF)呈负相关(rho = -0.86 和 -0.6),与 RVR 呈正相关(rho = 0.88)。uACR 的降低与肾脏弥散度的增加相关(rho = -0.5)。测量肾小球滤过率(GFR)与肾脏弥散度、RBF 和 FF 呈正相关(rho = 0.87、0.85 和 0.59),与 RVR 呈负相关(rho = -0.89);GFR 的增加与肾脏弥散度、RBF 和 FF 的增加(rho = 0.77、0.7 和 0.7)以及 RVR 的减少(rho = -0.7)相关。
尽管患者数量有限,但 MRI 与组织学和临床发现之间存在很强的相关性,这凸显了 MRI 监测罕见肾脏疾病患者疾病进展的潜力。