已知病因的门静脉-肝窦血管疾病有更严重的门静脉高压和更差的预后。

Porto-sinusoidal vascular disorder with known etiologies had more severe portal hypertension and poorer outcomes.

作者信息

Ma Lin, Zhang Guanhua, Wang Yu, Li Min, He Fuliang, Feng Lijuan, Wang Min, Liu Jimin, Zhao Xinyan, Jia Jidong

机构信息

Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China; State Key Laboratory of Digestive Health and National Clinical Research Center of Digestive Disease, Beijing, China.

Department of Clinical Epidemiology and EBM, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China.

出版信息

Dig Liver Dis. 2025 Mar;57(3):730-737. doi: 10.1016/j.dld.2024.10.025. Epub 2024 Nov 23.

DOI:10.1016/j.dld.2024.10.025

PMID:39581837

Abstract

BACKGROUND

Porto-sinusoidal vascular disorder (PSVD) is a clinicopathological entity and often associated with various etiologies. We aimed to compare the clinical and pathological features and outcomes of PSVD in patients with and without known etiologies in a Chinese cohort.

METHODS

This retrospective study enrolled liver-biopsy confirmed patients with PSVD. Patients' demographics, comorbidities, laboratory and imaging information were collected and the outcomes were followed up.

RESULTS

Totally 128 patients with PSVD were included, with 75 (58.6 %) having known etiologies including prothrombotic conditions, immunological disorders, hematological disorders, drug exposure. PSVD patients with known etiologies exhibited significantly higher rates of gastroesophageal varices (87.0 % vs 54.0 %, p < 0.001), portosystemic collaterals (94.0 % vs. 58.0 %, p < 0.001), lower level of serum albumin (36.5 vs 40.0, p < 0.001), and a higher prevalence of portal vein thrombosis (PVT, 34.0 % vs 13.0 %, p = 0.010). During follow-up, PSVD patients with known etiologies had a significantly higher incidence of liver-related events (ascites, variceal hemorrhage or HE) (p = 0.027).

CONCLUSIONS

PSVD with known etiologies had more severe portal hypertension, poorer liver function reserve, higher PVT prevalence, and worse clinical outcomes.

摘要

背景

门静脉窦状隙血管疾病（PSVD）是一种临床病理实体，常与多种病因相关。我们旨在比较中国队列中有已知病因和无已知病因的PSVD患者的临床、病理特征及预后。

方法

这项回顾性研究纳入了经肝活检确诊的PSVD患者。收集患者的人口统计学资料、合并症、实验室及影像学信息，并对预后进行随访。

结果

共纳入128例PSVD患者，其中75例（58.6%）有已知病因，包括血栓前状态、免疫性疾病、血液系统疾病、药物暴露。有已知病因的PSVD患者食管胃静脉曲张发生率显著更高（87.0%对54.0%，p<0.001），门体分流发生率更高（94.0%对58.0%，p<0.001），血清白蛋白水平更低（36.5对40.0，p<0.001），门静脉血栓形成（PVT）患病率更高（34.0%对13.0%，p=0.010）。随访期间，有已知病因的PSVD患者肝脏相关事件（腹水、静脉曲张出血或肝性脑病）发生率显著更高（p=0.027）。