Abstract：
It is well established that portal hypertension can occur in the absence of cirrhosis, as reported in patients with immune disorders, infections and thrombophilia. However, similar histological abnormalities primarily affecting the hepatic sinusoidal and (peri)portal vasculature have also been observed in patients without portal hypertension. Thus, the term porto-sinusoidal vascular disorder (PSVD) has recently been introduced to describe a group of vascular diseases of the liver featuring lesions encompassing the portal venules and sinusoids, irrespective of the presence/absence of portal hypertension. Liver biopsy is fundamental for PSVD diagnosis. Specific histology findings include nodular regenerative hyperplasia, obliterative portal venopathy/portal vein stenosis and incomplete septal fibrosis/cirrhosis. Since other conditions including alcohol-related and non-alcoholic fatty liver disease, or viral hepatitis, or the presence of portal vein thrombosis may occur in patients with PSVD, their relative contribution to liver damage should be carefully assessed. In addition to histology and clinical diagnostic criteria, imaging and non-invasive tests such as liver and spleen stiffness measurements could aid in the diagnostic workup. The introduction of PSVD as a novel clinical entity will facilitate collaborative studies and investigations into the underlying molecular pathomechanisms encompassed by this term.
摘要：
众所周知，门脉高压可以在没有肝硬化的情况下发生，据报道，在免疫疾病患者中，感染和血栓形成倾向。然而,在没有门静脉高压症的患者中也观察到主要影响肝窦和（周围）门静脉系统的类似组织学异常。因此，最近引入了术语门窦血管疾病（PSVD）来描述一组肝脏血管疾病，其特征是病变包括门静脉和窦状，无论是否存在门静脉高压症。肝活检是PSVD诊断的基础。具体的组织学发现包括结节性再生增生，闭塞性门静脉病/门静脉狭窄和不完全性间隔纤维化/肝硬化。由于其他条件，包括酒精相关和非酒精性脂肪肝疾病，或者病毒性肝炎，或者在PSVD患者中可能发生门静脉血栓形成，应仔细评估它们对肝损伤的相对贡献。除了组织学和临床诊断标准，影像学检查和非侵入性检查，如肝脏和脾脏硬度测量，可能有助于诊断检查。PSVD作为一种新型临床实体的引入将促进对该术语所涵盖的潜在分子病理机制的合作研究和调查。
