University Clinic for Visceral Surgery and Medicine, Inselspital, Bern, Switzerland; Department of Biomedical Research, University of Bern, Bern, Switzerland.
Service d'Hépatologie, Hôpital Beaujon, Clichy, France; Centre de Recherche de l'Inflammation, Inserm and Université Paris Diderot, Paris, France.
Lancet Gastroenterol Hepatol. 2019 May;4(5):399-411. doi: 10.1016/S2468-1253(19)30047-0.
Portal hypertension in the absence of portal vein thrombosis and without cirrhosis, but with mild or moderate alterations of liver histology (eg, obliterative venopathy, nodular regenerative hyperplasia, or incomplete septal cirrhosis) is being increasingly recognised. Owing to the heterogeneity of causes and histological findings, a substantial number of terms have been used to describe such idiopathic non-cirrhotic portal hypertension. Patients with the same clinical and histological features exist, but without portal hypertension at the time of diagnosis. Therefore, improved criteria are needed to define this form of liver disease. Here, we propose the term porto-sinusoidal vascular disease, since all lesions found involve the portal venules or sinusoids. The definition of this entity is based on the characteristic absence of cirrhosis with or without signs of portal hypertension or histological lesions. The presence of known causes of liver disease does not rule out porto-sinusoidal vascular disease, but specific causes of vascular liver disease are excluded from its definition. The diagnosis of porto-sinusoidal vascular disease is based on liver biopsy and might include signs specific for portal hypertension with normal or mildly elevated liver stiffness values and no complete portal vein thrombosis. We provide simple diagnostic criteria, because agreement on a uniform nomenclature is an essential requirement for future collaborative studies.
在不存在门静脉血栓且无肝硬化的情况下，伴有轻微或中度肝组织学改变（如闭塞性静脉病、结节性再生性增生或不完全间隔性肝硬化）的门静脉高压症正日益受到关注。由于病因和组织学发现的异质性，已经使用了大量术语来描述这种特发性非肝硬化性门静脉高压症。存在具有相同临床和组织学特征的患者，但在诊断时没有门静脉高压。因此，需要改进标准来定义这种形式的肝病。在这里，我们提出了门腔静脉血管病的术语，因为所有发现的病变都涉及门静脉小静脉或窦。该实体的定义基于特征性的无肝硬化，伴有或不伴有门静脉高压或组织学病变的存在。已知肝病的病因并不排除门腔静脉血管病，但将特定的血管性肝病病因排除在其定义之外。门腔静脉血管病的诊断基于肝活检，可能包括具有正常或轻度升高的肝硬度值且无完全门静脉血栓形成的门静脉高压的特征性表现。我们提供了简单的诊断标准，因为统一命名法的共识是未来合作研究的基本要求。
