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De Gottardi_2022_Else_joh
Journal article

Porto-sinusoidal vascular disorder

  • De Gottardi, Andrea ORCID Gastroenterology and Hepatology, Ente Ospedaliero Cantonale, Lugano, Switzerland - Faculty of Biomedical Sciences, Università della Svizzera Italiana, Lugano, Switzerland
  • Sempoux, Christine ORCID Institute of Pathology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
  • Berzigotti, Annalisa ORCID Department for Visceral Medicine and Surgery, Inselspital, Bern University Hospital, University of Bern, Switzerland - Department of Biomedical Research, University of Bern, Bern, Switzerland
  • 2022
Published in:
  • Journal of Hepatology. - 2022, vol. 77, no. 4, p. 1124–1135
Obliterative portal venopathy
Nodular regenerative hyperplasia
Incomplete septal fibrosis
Portal vein stenosis
Idiopathic noncirrhotic portal hypertension
English 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.
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Language
  • English
Classification
Medicine
License
CC BY
Open access status
green
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Persistent URL
https://n2t.net/ark:/12658/srd1322474
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