Liu Jie, Zhang Qian, Liu Yao, Ma Hai-Xia, Han Xu, Ma Ying, Zhao Li-Li, Li Jia
Department of Hepatology, Tianjin Second People's Hospital, Tianjin 300192, China.
Clinical School of the Second People's Hospital, Tianjin Medical University, Tianjin 300070, China.
Diagnostics (Basel). 2024 Sep 16;14(18):2053. doi: 10.3390/diagnostics14182053.
Porto-sinusoidal vascular disease (PSVD) as a novel clinical conception was modified on the basis of idiopathic non-cirrhotic portal hypertension (INCPH). This study aimed to compare the clinical, biochemical histological features and prognosis between the diagnostic criteria for PSVD and that of INCPH.
A total of 65 patients who underwent liver biopsies were analyzed retrospectively. The clinical, pathological and prognosis date were reviewed and screened according to the latest diagnostic criteria of PSVD and INCPH.
A total of 65 patients were diagnosed with PSVD, of which 31 (47.69%) also fulfilled INCPH criteria. Specific histological and specific clinical portal hypertension (PH) signs were found in 34 (52.31%) and 30 (46.15%) of the patients, respectively. PSVD patients showed higher LSM levels (11.45 (6.38, 18.08) vs. 7.90 (6.70, 13.00), = 0.039) than the INCPH patients. INCPH patients had a higher cumulative incidence of liver-related complications than the PSVD patients (86.95% vs. 35.71%, log-rank < 0.001).
Novel PSVD criteria facilitate early diagnosis. PSVD patients with other liver diseases may have higher LSM values. Disease progression and survival outcomes are correlated with PH in PSVD patients.
肝窦状隙血管病（PSVD）作为一种新的临床概念，是在特发性非肝硬化门静脉高压症（INCPH）的基础上修订而来。本研究旨在比较PSVD和INCPH诊断标准之间的临床、生化组织学特征及预后。
回顾性分析65例行肝活检的患者。根据PSVD和INCPH的最新诊断标准，对临床、病理及预后数据进行回顾和筛选。
65例患者被诊断为PSVD，其中31例（47.69%）也符合INCPH标准。分别在34例（52.31%）和30例（46.15%）患者中发现了特异性组织学和特异性临床门静脉高压（PH）体征。PSVD患者的肝脏硬度值（LSM）水平高于INCPH患者[11.45（6.38，18.08）对7.90（6.70，13.00），P = 0.039]。INCPH患者肝脏相关并发症的累积发生率高于PSVD患者（86.95%对35.71%，对数秩检验P<0.001）。
新的PSVD标准有助于早期诊断。合并其他肝脏疾病的PSVD患者可能具有更高的LSM值。PSVD患者的疾病进展和生存结局与PH相关。
