Disclosures: Pere Gines – Advisory Committees or Review Panels: Ferring; Grant/Research Support: Sequana Medical, Grifols Rajiv Jalan – Consulting: Ocera Therapeutics, Conatus Juan Cordoba – Advisory Committees or Review Panels: Ocera Francois Durand – Advisory Committees or Review Panels: Astellas, Novartis; Speaking and Teaching: Gilead Faouzi Saliba Epigenetics inhibitor – Advisory Committees or Review Panels: Novartis, Roche, Genzyme; Grant/Research Support: Astellas; Speaking and Teaching: Schering Plough, Gambro, MSD Julia Wendon – Consulting: Pulsion, Excalenz Stefan Zeuzem – Consulting: Abbvie, Achillion Pharmaceuticals, Boehringer Ingelheim GmbH, Bristol-Myers Squibb Co., Gilead, Novartis Pharmaceuticals,

Merck & Co., Idenix, Janssen, Roche Pharma AG, Vertex Pharmaceuticals, Presidio, Santaris, Inc Mauro Bernardi – Consulting: CLS Behring GhmB; Speaking and Teaching: CLS Behring GhmB, PPTA Europe Vicente Arroyo – Speaking and Teaching: GRIFOLS The following people have nothing to disclose: Paolo Angeli, Salvatore Piano, Elisabet Garcίa, Filippo Morando, Ezequiel Rodriguez, Xavier Ariza, Elisabetta Gola, Elsa Solá, Monica Guevara, Richard Moreau, Marco Pavesi, Thierry Gustot, Marco Domenicali, Alexander L. Gerbes, Carlo Alessandria, Wim Laleman, Jonel Trebicka Background:

Symptomatic liver disease in hereditary hemorrhagic telangiectasia (HHT) is rare but may be fatal without liver transplantation. Aims: To identify risk factors predictive of clinically significant liver disease. Methods: In this prospective cohort study, we included consecutive patients referred to our HHT center from 2001 learn more with definite HHT. Patients underwent systematic protocol screening, including contrast-enhanced CT. From a multivariable logistic regression model, we

developed a simple clinical scoring index that may predict presence of clinically selleck compound significant liver disease [symptomatic liver disease (cardiac failure, portal hypertension or biliary disease) or at-risk liver involvement with abnormal liver examination (liver bruit, thrill, palpable hepatomegaly) or biochemistry or elevated cardiac index]. Results: Three hundred seventeen patients with definite HHT were included; 171 patients (54.5%; age 50.7±14.7 y, 101 females) had hepatic involvement on imaging. Twenty nine patients had symptomatic liver disease (22 with high-output heart failure, 6 with portal hypertension and 3 with symptomatic biliary disease) and 45 patients had at-risk liver disease. Ninety seven patients (56.7%) had hepatic involve-ment without symptomatic or at-risk liver disease. Using multivariable logistic regression analysis, four clinical variables (age, gender, hemoglobin and alkaline phosphatase) were modeled to develop a simple clinical scoring index (c-statistic to distinguish clinically significant liver involvement and no or incidental liver lesions=0.83). Table shows the probability of clinically significant liver disease based on risk score.