Clinical Research Center for Rare Diseases "Aldo & Cele Daccò", Istituto Di Ricerche Farmacologiche Mario Negri IRCCS, Via Camozzi 3, 24020, Bergamo, Ranica, Italy.
Department of Diagnostic Radiology, Azienda Socio-Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy.
Eur Radiol. 2023 Sep;33(9):6009-6019. doi: 10.1007/s00330-023-09601-4. Epub 2023 Apr 5.
Beyond total kidney and cyst volume (TCV), non-cystic tissue plays an important role in autosomal dominant polycystic kidney disease (ADPKD) progression. This study aims at presenting and preliminarily validating a diffusion MRI (DWI)-based TCV quantification method and providing evidence of DWI potential in characterising non-cystic tissue microstructure.
T2-weighted MRI and DWI scans (b = 0, 15, 50, 100, 200, 350, 500, 700, 1000; 3 directions) were acquired from 35 ADPKD patients with CKD stage 1 to 3a and 15 healthy volunteers on a 1.5 T scanner. ADPKD classification was performed using the Mayo model. DWI scans were processed by mono- and segmented bi-exponential models. TCV was quantified on T2-weighted MRI by the reference semi-automatic method and automatically computed by thresholding the pure diffusivity (D) histogram. The agreement between reference and DWI-based TCV values and the differences in DWI-based parameters between healthy and ADPKD tissue components were assessed.
There was strong correlation between DWI-based and reference TCV (rho = 0.994, p < 0.001). Non-cystic ADPKD tissue had significantly higher D, and lower pseudo-diffusion and flowing fraction than healthy tissue (p < 0.001). Moreover, apparent diffusion coefficient and D values significantly differed by Mayo imaging class, both in the whole kidney (Wilcoxon p = 0.007 and p = 0.004) and non-cystic tissue (p = 0.024 and p = 0.007).
DWI shows potential in ADPKD to quantify TCV and characterise non-cystic kidney tissue microstructure, indicating the presence of microcysts and peritubular interstitial fibrosis. DWI could complement existing biomarkers for non-invasively staging, monitoring, and predicting ADPKD progression and evaluating the impact of novel therapies, possibly targeting damaged non-cystic tissue besides cyst expansion.
This study shows diffusion-weighted MRI (DWI) potential to quantify total cyst volume and characterise non-cystic kidney tissue microstructure in ADPKD. DWI could complement existing biomarkers for non-invasively staging, monitoring, and predicting ADPKD progression and evaluating the impact of novel therapies, possibly targeting damaged non-cystic tissue besides cyst expansion.
• Diffusion magnetic resonance imaging shows potential to quantify total cyst volume in ADPKD. • Diffusion magnetic resonance imaging might allow to non-invasively characterise non-cystic kidney tissue microstructure. • Diffusion magnetic resonance imaging-based biomarkers significantly differ by Mayo imaging class, suggesting their possible prognostic value.
除总肾脏和囊肿体积(TCV)外,非囊肿组织在常染色体显性多囊肾病(ADPKD)进展中也起着重要作用。本研究旨在提出并初步验证一种基于弥散磁共振成像(DWI)的 TCV 定量方法,并提供 DWI 用于描述非囊肿组织微观结构的潜力的证据。
在 1.5T 扫描仪上对 35 名患有 CKD 1 至 3a 期的 ADPKD 患者和 15 名健康志愿者进行了 T2 加权 MRI 和 DWI 扫描(b=0、15、50、100、200、350、500、700、1000;3 个方向)。使用 Mayo 模型对 ADPKD 进行分类。DWI 扫描通过单指数和分段双指数模型进行处理。在 T2 加权 MRI 上通过参考半自动方法定量 TCV,并通过对纯弥散度(D)直方图进行阈值处理自动计算 TCV。评估了参考和基于 DWI 的 TCV 值之间的一致性,以及健康组织和 ADPKD 组织成分之间基于 DWI 的参数之间的差异。
基于 DWI 的 TCV 值与参考值之间具有很强的相关性(rho=0.994,p<0.001)。与健康组织相比,非囊肿性 ADPKD 组织的 D 值更高,假性扩散和流动分数更低(p<0.001)。此外,表观扩散系数和 D 值在整个肾脏(Wilcoxon p=0.007 和 p=0.004)和非囊肿组织(p=0.024 和 p=0.007)中均根据 Mayo 成像分类而显著不同。
DWI 显示出在 ADPKD 中定量 TCV 和描述非囊肿性肾脏组织微观结构的潜力,表明存在微小囊肿和肾小管周围间质纤维化。DWI 可以补充现有的生物标志物,用于非侵入性分期、监测和预测 ADPKD 的进展,并评估新型疗法的影响,可能除了囊肿扩张外,还可以针对受损的非囊肿组织。
本研究表明,扩散加权磁共振成像(DWI)有可能定量测定 ADPKD 中的总囊肿体积并描绘非囊肿性肾脏组织的微观结构。DWI 可以补充现有的生物标志物,用于非侵入性分期、监测和预测 ADPKD 的进展,并评估新型疗法的影响,可能除了囊肿扩张外,还可以针对受损的非囊肿组织。
弥散磁共振成像显示出在 ADPKD 中定量总囊肿体积的潜力。
弥散磁共振成像可能允许对非囊肿性肾脏组织微观结构进行非侵入性描述。
基于弥散磁共振成像的生物标志物根据 Mayo 成像分类显著不同,表明其具有预测价值。