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

De Gottardi, Andrea; Rautou, Pierre-Emmanuel; Schouten, Jeoffrey; Rubbia-Brandt, Laura; Leebeek, Frank; Trebicka, Jonel; Murad, Sarwa Darwish; Vilgrain, Valérie; Hernandez-Gea, Virginia; Nery, Filipe; Plessier, Aurélie; Berzigotti, Annalisa; Bioulac-Sage, Paulette; Primignani, Massimo; Semela, David; Elkrief, Laure; Bedossa, Pierre; Valla, Dominique; Garcia-Pagan, Juan Carlos (2019). Porto-sinusoidal vascular disease: proposal and description of a novel entity. The lancet. Gastroenterology & hepatology, 4(5), pp. 399-411. Elsevier 10.1016/S2468-1253(19)30047-0

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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.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > DBMR Forschung Mu35 > Forschungsgruppe Hepatologie
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > DBMR Forschung Mu35 > Forschungsgruppe Hepatologie

04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Visceral Surgery and Medicine > Hepatology

UniBE Contributor:

De Gottardi, Andrea and Berzigotti, Annalisa

Subjects:

600 Technology
600 Technology > 610 Medicine & health

ISSN:

2468-1253

Publisher:

Elsevier

Language:

English

Submitter:

Thi Thao Anh Pham

Date Deposited:

30 Jan 2020 14:37

Last Modified:

19 Mar 2020 14:15

Publisher DOI:

10.1016/S2468-1253(19)30047-0

PubMed ID:

30957754

BORIS DOI:

10.7892/boris.137260

URI:

https://boris.unibe.ch/id/eprint/137260

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