The epidemiology of Budd–Chiari syndrome in France

Isabelle Ollivier-Hourmand (1) , Manon Allaire (1, 2) , Nathalie Goutte , Rémy Morello , Carine Chagneau-Derrode (3) , Odile Goria (4) , Jérôme Dumortier (5) , Jean Paul Cervoni (6) , Sébastien Dharancy (7, 8) , Nathalie Ganne-Carrié (9) , Christophe Bureau , Nicolas Carbonell (10) , Armand Abergel (11) , Jean Baptiste Nousbaum (12) , Rodolphe Anty , Helene Barraud (13) , Marie Pierre Ripault , Victor de Ledinghen (14) , Anne Minello (15) , Frédéric Oberti (16) , Sylvie Radenne (17) , Noelle Bendersky (18) , Olivier Farges (19) , Isabelle Archambeaud (20) , Anne Guillygomarc'H , Marie Ecochard (21) , Violaine Ozenne (22) , Marie Noëlle Hilleret (18) , Noelle Hilleret (18) , Eric Nguyen-Khac (23) , Barbara Dauvois (24) , Jean Marc Perarnau (25) , Pascale Lefilliatre , Jean Jacques Raabe (26) , Michel Doffoel (27) , Jean Philippe Becquart , Eric Saillard (28) , Dominique Valla , Thong Dao (1) , Aurélie Plessier (19) , - French Network For Vascular Disorders of The Liver

1 Service d'Hépato-Gastro-Enterologie et Nutrition [CHU Caen]
2 CRI (UMR_S_1149 / ERL_8252 / U1149) - Centre de recherche sur l'Inflammation
3 Service d’hépato-gastro-entérologie et assistance nutritive [CHU de Poitiers]
4 Service d'Hépato-Gastroentérologie [CHU Rouen]
5 Service d'hépatogastroentérologie [Hôpital Edouard Herriot, Hospices Civils de Lyon]
6 Service d'Hépatologie [CHRU Besançon]
7 Service d'Hépato-gastroentérologie [CHU Lille]
8 LIRIC - Lille Inflammation Research International Center - U 995
9 U1162 - Génomique Fonctionnelle des Tumeurs Solides
10 CHU Saint-Antoine [AP-HP]
11 Service d'Hépatologie Gastro-entérologie [CHU Clermont-Ferrand]
12 Service d'Hépato-Gastroentérologie [CHRU Brest]
13 Service d'Hépato-gastro-entérologie [CHRU Nancy]
14 Service d'Hépato-gastro-entérologie et oncologie digestive (CHU de Bordeaux)
15 Service d'Hépato-Gastro-Entérologie (CHU de Dijon)
16 Département d'hépatologie (CHU Angers)
17 Service d'Hépatologie [Hôpital de la Croix-Rousse - HCL]
18 Service d'hépato-gastroentérologie [CHU Grenoble Alpes]
19 Service d’Hépatologie [Hôpital Beaujon]
20 Département d'hépato-gastro-entérologie [CHU Nantes]
21 Service de Gastro-entérologie et Hépatologie [CHU Saint-Etienne]
22 Hôpital Lariboisière-Fernand-Widal [APHP]
23 Service d'Hépato-gastroentérologie [CHU Amiens-Picardie]
24 Service d'hépato-gastro-entérologie et oncologie digestive [CHR Orléans]
25 Département d'Hépato-Gastroentérologie [Hôpital Trousseau, Tours]
26 Service d'Hépato-gastro-entérologie [CHR Metz-Thionville]
27 Département d'hépato-gastro-entérologie et d'assistance nutritionnelle [CHU Strasbourg]
28 CHU Pointe-à-Pitre/Abymes [Guadeloupe]

Introduction Epidemiological data is lacking on primary Budd–Chiari syndrome (BCS) in France. Methods Two approaches were used: (1) A nationwide survey in specialized liver units for French adults. (2) A query of the French database of discharge diagnoses screening to identify incident cases in adults. BCS associated with cancer, alcoholic/viral cirrhosis, or occurring after liver transplantation were classified as secondary. Results Approach (1) 178 primary BCS were identified (prevalence 4.04 per million inhabitants (pmi)), of which 30 were incident (incidence 0.68 pmi). Mean age was 40 ± 14 yrs. Risk factors included myeloproliferative neoplasms (MPN) (48%), oral contraceptives (35%) and factor V Leiden (16%). None were identified in 21% of patients, ≥2 risk factors in 25%. BMI was higher in the group without any risk factor (25.7 kg/m2 vs 23.7 kg/m2, p < 0.001). Approach (2) 110 incident primary BCS were admitted to French hospitals (incidence 2.17 pmi). MPN was less common (30%) and inflammatory local factors predominated (39%). Conclusion The entity of primary BCS as recorded in French liver units is 3 times less common than the entity recorded as nonmalignant hepatic vein obstruction in the hospital discharge database. The former entity is mostly related to MPN whereas the latter with abdominal inflammatory diseases.

Mots clés: Hepatic vein obstruction; Incidence; National database; Questionnaire survey; Risk factors

Domaines: Hépatologie et Gastroentérologie

Isabelle Ollivier-Hourmand, Manon Allaire, Nathalie Goutte, Rémy Morello, Carine Chagneau-Derrode, et al.. The epidemiology of Budd–Chiari syndrome in France. Digestive and Liver Disease, 2018, 50 (9), pp.931-937. ⟨10.1016/j.dld.2018.04.004⟩. ⟨hal-02057088⟩