Porto-sinusoidal vascular disorder
Additional information
Authors
De Gottardi A.,
Sempoux C.,
Berzigotti A.
Type
Journal Article
Year
2022
Language
English
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.
Keywords
Obliterative portal venopathy, Nodular regenerative hyperplasia, Incomplete septal fibrosis, Portal vein stenosis, Idiopathic noncirrhotic portal hypertension
Journal
Journal of Hepatology
Volume
77
Number ( Month )
4
Pages (or article number)
1124–1135
Diffusion
License
CC BY
Visibility
Public
Status open access
Hybrid
