• A Riescher-Tuczkiewicz, L Elkrief, and P-E Rautou.
• Université Paris-Cité, Inserm, centre de recherche sur l'inflammation, UMR 1149, Paris, France. Electronic address: Alix.riescher-tuczkiewicz@inserm.fr.
• Rev Med Interne. 2024 Jan 1; 45 (1): 172517-25.

AbstractSplanchnic vein thrombosis includes Budd-Chiari syndrome and portal vein thrombosis. These diseases share common features: (i) they are rare diseases and (ii) they can lead to portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, the most common being myeloproliferative neoplasms. A rapid and comprehensive workup for thrombosis risk factors is necessary in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis, and is associated with a worse course of cirrhosis. Indications for anticoagulation in patients with cirrhosis are increasing. Transjugular intrahepatic portosystemic shunt is a second-line procedure in this setting. Because of the rarity of these diseases, high-level evidence studies are rare. However, collaborative studies have provided a better understanding of their natural history and allowed to improve the management of these patients. This review focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, patients with portal vein thrombosis without underlying liver disease, and patients with cirrhosis and portal vein thrombosis.Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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