Porto-sinusoidal vascular disease: a view through a new paradigm. Lecture

The lecture presents the current data on porto‑sinusoidal vascular disease (PSVD), which is a rare vascular disease of the liver that, in the vast majority of cases, is accompanied by the development of portal hypertension in the absence of liver cirrhosis and is mostly underdiagnosed due to a lack of knowledge about this pathology. Brief historical information on the sequence of study of PSVD and changes in the understanding of the root causes of this pathology are presented. The lack of unanimous opinion of the leading hepatological societies — Baveno VII, European Association for the Study of the Liver (EASL), Vascular Liver Diseases Interest Group (VALDIG), American Association for the Study of Liver Diseases (AASLD), Asian Pacific Association for the Study of the Liver (APASL) — on the terminology and management of patients with PSVD is emphasized. Modern diagnostic criteria for VALDIG are described, and the characteristic features detected during instrumental imaging studies (absence of nodular changes on the liver surface, hypertrophy of segments IV and I against the background of unchanged or slightly increased liver stiffness along with signs of portal hypertension) are noted. It is emphasized that a mandatory requirement for the diagnosis of PSVD is a liver biopsy and the detection of some specific histological signs of the disease (obliterating portal venopathy, nodular regenerative hyperplasia, incomplete septal fibrosis). It is noted that the course of PSVD is complicated by bleeding from varicose veins, ascites, and hepatic encephalopathy. Current views on the treatment of patients with PSVD are similar to the management of patients with cirrhosis: therapy is aimed at combating complications of portal hypertension and preventing their occurrence. Opposing views on the long‑term prognosis of PSVD are presented: in general, it is considered relatively favorable, but it can worsen with the development of concomitant portal hypertension.