Department of Neurology, Seoul National University Hospital, Seoul, South Korea.
Department of Neurology, Seoul National University College of Medicine, Seoul, South Korea.
J Cereb Blood Flow Metab. 2021 Dec;41(12):3365-3377. doi: 10.1177/0271678X211037869. Epub 2021 Aug 20.
Cilia dysfunction in autosomal-dominant polycystic kidney disease (ADPKD) may impair the integrity of glymphatic system and be implicated in the progression of cerebral small vessel disease (SVD), although the link between the two diseases has not been investigated. We evaluated the association of ADPKD pathology with SVD pattern and severity. Overall, 304 individuals in an ADPKD (chronic kidney disease stage ≤4 and age ≥50 years) cohort and their age, sex, and estimated glomerular filtration rate (eGFR)-matched controls were retrospectively included. ADPKD severity was classified into 1 A-B, 1 C, and 1 D-E, according to age and height-adjusted total kidney volume. SVD parameters included white-matter hyperintensity (WMH) severity scale, enlarged perivascular space (ePVS) score, and degree of lacunes or cerebral microbleeds (CMBs). After adjustments for age, sex, eGFR, and cerebrovascular risk factor parameters, ADPKD was associated with higher ePVS scores ( < 0.001), but not with the WMH severity or degree of lacunes or CMBs. In the ADPKD subgroup, higher ADPKD severity class was associated with higher ePVS scores ( < 0.001), WMH severity ( = 0.003), and degree of lacunes ( = 0.002). ADPKD associated cilia dysfunction may induce chronic cerebral glymphatic system dysfunction, which may contribute to the specific progression of ePVS compared with other SVD markers.
常染色体显性多囊肾病 (ADPKD) 中的纤毛功能障碍可能会损害糖质系统的完整性，并与脑小血管疾病 (SVD) 的进展有关，尽管这两种疾病之间的联系尚未得到研究。我们评估了 ADPKD 病理学与 SVD 模式和严重程度之间的关系。总体而言，回顾性纳入了 304 名 ADPKD（慢性肾脏病分期≤4 且年龄≥50 岁）患者及其年龄、性别和估算肾小球滤过率 (eGFR) 匹配的对照者。根据年龄和身高校正的总肾脏体积，将 ADPKD 严重程度分为 1A-B、1C 和 1D-E。SVD 参数包括白质高信号 (WMH) 严重程度量表、扩大的血管周围间隙 (ePVS) 评分以及腔隙或脑微出血 (CMBs) 的程度。在调整年龄、性别、eGFR 和脑血管危险因素参数后，ADPKD 与更高的 ePVS 评分相关（<0.001），但与 WMH 严重程度或腔隙或 CMBs 程度无关。在 ADPKD 亚组中，更高的 ADPKD 严重程度分级与更高的 ePVS 评分（<0.001）、WMH 严重程度（=0.003）和腔隙程度（=0.002）相关。ADPKD 相关纤毛功能障碍可能会引起慢性脑糖质系统功能障碍，这可能导致与其他 SVD 标志物相比，ePVS 具有特定的进展。
