Assistance Publique-Hôpitaux de Paris, Hôpital Beaujon

Particular rare liver disease expertise of the centre

Vascular liver diseases (VLD) :

• Budd-Chiari syndrome (BCS)
• Non-cirrhotic acute and chronic portal vein thrombosis (PVT),
• Portal cavernoma or chronic non cirrhotic extrahepatic portal vein obstruction,
• Extrahepatic portal vein obstruction (ehpvo) in cirrhosis
• Veno occlusive disease (VOD),
• Sinusoidal obstruction syndrome (SOS)
• Idiopathic non-cirrhotic portal hypertension (INCPH),
• Porto sinusoidal vascular disease,
• Drug induced liver injury and vascular liver disease,
• Ischemic cholangiopathy,
• Congenital portosystemic shunts,
• Pregnancy and vascular liver diseases,
• Fontan related liver disease

Risk Factors for Vascular Liver Diseases 
Management and treatments for VLD:

• Percutaneous liver biopsy
• Transjugular liver biopsy
• Hepatic hemodynamic studies
• Direct transhepatic venography,
• Retrograde transjugular or transfemoral venography
• Anticoagulation therapy in patients at risk for gastrointestinal bleeding related to portal hypertension
• Percutaneous angioplasty and stenting
• Liver transplantation

French network competence center for Autoimmune and biliary diseases:

• Primary Biliary Cholangitis (PBC, formerly Primary Biliary Cirrhosis)
• Autoimmune Hepatitis (AIH)
• Primary Sclerosing Cholangitis (PSC)
• Alpha-1 Antitrypsin Liver Disease
• Wilson’s Disease
• LPAC syndrome

French network competence center for biliary atresia and intrahepatic cholestasis

• Benign recurrent intrahepatic cholestasis
• Familial progressive intrahepatic cholestasis Biliary Atresia
• Choledochal Cyst, Alagille Syndrome

ERN Team and Contact

• Dr Odile GORIA,
• Dr Aurélie PLESSIER
• Dr Audrey PAYANCE
• Pr Pierre-Emmanuel RAUTOU
• Pr Dominique VALLA
• Nurse Coordinator – Mrs Marie SANTIN – marie.santin(at)aphp.fr
• Assistant – Mrs Valérie de BREMAND – valerie.debremand(at)aphp.fr
• Clinical Research Associate – Mr Kamal Zékrini – kamal.zekrini(at)aphp.fr

Centre information

The Referral Centre for Vascular Liver Diseases in France “Centre de Référence des Maladies Vasculaires du Foie” (CRMVF) coordinates the national network for vascular liver diseases, which includes 32 local competence centres and 2 constitutive reference centres (Bicêtre, Tours). The network was accredited by the French Ministry of Health in 2005. The accreditation by the French Ministry of Health was renewed in 2023.

The CRMVF, coordinated by Dr Plessier at Hôpital Beaujon, is one of the four centres of the French rare liver disease network FILFOIE (filière for rare liver diseases), accredited in 2015. It is also part of the academic department DHU UNITY (addressing the Unmet Needs through Innovation in gastroenterology and hepatologY), accredited in 2014.

CRMVF is a patient-centred care network that aims to offer the best possible management for patients with vascular liver diseases, regardless of where they live in France. The multidisciplinary team includes experts in vascular liver diseases, hepatology and liver transplantation, diagnostic and interventional radiology, endoscopy, haematology, biochemistry, intensive care and anaesthesiology, as well as liver and gastrointestinal surgery. The goal is to provide expert management as locally as possible.

Only when necessary — for example, for technical procedures that cannot be performed elsewhere — are patients referred to the CRMVF at Hôpital Beaujon. This model fosters improved local management through training and close collaboration.

CRMVF’s two additional aims are:

• to share best practices, nationally and internationally, in the management of patients
• to foster and expand clinical and translational research

The centre is also a competence centre for autoimmune and biliary diseases coordinated by Dr Payancé and biliary atresia and cholestatic liver diseases coordinated by Dr Plessier.