Porto-sinusoidal vascular disease: proposal and description of a novel entity

VALDIG group, Andrea De Gottardi, Pierre Emmanuel Rautou, Jeoffrey Schouten, Laura Rubbia-Brandt, Frank Leebeek, Jonel Trebicka, Sarwa Darwish Murad, Valérie Vilgrain, Virginia Hernandez-Gea, Filipe Nery, Aurélie Plessier, Annalisa Berzigotti, Paulette Bioulac-Sage, Massimo Primignani, David Semela, Laure Elkrief, Pierre Bedossa, Dominique Valla, Juan Carlos Garcia-Pagan

Research output: Contribution to journalComment/debatepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)399-411
Number of pages13
JournalThe Lancet Gastroenterology and Hepatology
Volume4
Issue number5
DOIs
Publication statusPublished - May 1 2019

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

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