TY - JOUR
T1 - Porto-sinusoidal vascular disease
T2 - proposal and description of a novel entity
AU - VALDIG group
AU - De Gottardi, Andrea
AU - Rautou, Pierre Emmanuel
AU - Schouten, Jeoffrey
AU - Rubbia-Brandt, Laura
AU - Leebeek, Frank
AU - Trebicka, Jonel
AU - Murad, Sarwa Darwish
AU - Vilgrain, Valérie
AU - Hernandez-Gea, Virginia
AU - Nery, Filipe
AU - Plessier, Aurélie
AU - Berzigotti, Annalisa
AU - Bioulac-Sage, Paulette
AU - Primignani, Massimo
AU - Semela, David
AU - Elkrief, Laure
AU - Bedossa, Pierre
AU - Valla, Dominique
AU - Garcia-Pagan, Juan Carlos
PY - 2019/5/1
Y1 - 2019/5/1
N2 - 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.
AB - 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.
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U2 - 10.1016/S2468-1253(19)30047-0
DO - 10.1016/S2468-1253(19)30047-0
M3 - Comment/debate
AN - SCOPUS:85063735164
VL - 4
SP - 399
EP - 411
JO - The Lancet Gastroenterology and Hepatology
JF - The Lancet Gastroenterology and Hepatology
SN - 2468-1253
IS - 5
ER -