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Study Protocol: The natural history of early porto-sinusoidal vascular disorder

Jun 13, 2023

Dear all,

We would like to launch a new study entitled “The natural history of early porto-sinusoidal vascular disorder” within the VALDIG network.

Currently, there is a gap in knowledge regarding the natural history of patients with histological lesions of PSVD and without signs of portal hypertension. In this study, we will aim to describe the natural history of early stages of PSVD (without signs of portal hypertension) and to clarify risk factors for disease progression. We believe that this study is needed and will help to better understand the natural history of this disease.

Please do not hesitate to contact us if you want additional information.

Best regards,

Edeline Kaze & Pierre Deltenre

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Aims

Porto-sinusoidal vascular disorder (PSVD) is an entity that regroups several mild or moderate
alterations of liver histology in the absence of cirrhosis. It may be associated with portal
hypertension despite no portal vein obstruction is observed (1,2). According to a recent review
article, PSVD is diagnosed when a liver biopsy >20 mm rules out cirrhosis and when there is
one specific sign of portal hypertension (gastric, esophageal or ectopic varices, portal
hypertensive bleeding, porto-systemic collaterals at imaging) or one specific histological lesion
for PSVD (obliterative portal venopathy, nodular regenerative hyperplasia, incomplete septal
fibrosis). In the absence of specific signs of portal hypertension and specific histological lesions,
PSVD can be diagnosed when there is one non-specific sign of portal hypertension (ascites,
platelet count < 150’000/mm3, spleen size ≥13 cm in the largest axis) and one non-specific
histological lesion for PSVD (portal tract abnormalities, architectural disturbance: irregular
distribution of the portal tracts and central veins, non-zonal sinusoidal dilatation, mild

°To describe the natural history of the early stages of PSVD without signs of portal hypertension

°To clarify risk factors for disease progression

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