title: Bevacizumab to Treat Cholangiopathy in Hereditary Hemorrhagic Telangiectasia: Be Cautious: A Case Report.
abstract: Hereditary hemorrhagic telangiectasia (HHT) is an inherited vascular dysplasia characterized by mucocutaneous telangiectasia and visceral arteriovenous malformations. Hepatic involvement with vascular malformations may lead to portal hypertension, biliary ischemia, and high-output cardiac failure. There is no curative treatment for the disease. Liver transplantation is indicated for life-threatening complications, but it carries significant risk due to surgery and immunosuppressive treatment. Some case reports or small open studies suggest that bevacizumab, a recombinant humanized anti-VEGF monoclonal antibody, should be efficient in limiting bleeding and in reducing liver disease in HHT.We report a case of a 63-year-old woman with HHT presenting with ischemic cholangiopathy. Liver transplant was indicated, but given a previous encouraging report showing a regression of biliary disease with bevacizumab in 3 patients with HHT this drug was proposed. No significant efficacy but a severe adverse effect was observed after 3 months: bilateral pulmonary embolisms, thrombosis in the right atrial cavity, and thrombosis of the right hepatic vein were evidenced. Bevacizumab was stopped; anticoagulant started. Four months later, the patient received a transplanted liver. She feels well 1 year later.This case report intends to provide the information for clinicians to consider the use of bevacizumab in HHT. Whereas several uncontrolled series and case reports have suggested the efficacy of this drug in reducing bleeding and liver disease, no severe side effects were mentioned to date. For the first time in HHT we report a life-threatening side effect of this drug and no efficacy. Moreover, systemic thrombosis, the observed complication, may preclude transplantation. To date, caution seems still indispensable when considering the use of bevacizumab in HHT.
authors.affiliation: From the Department of Internal Medicine, Infectious diseases and Clinical Immunology, Robert-Debré Hospital, Université de Reims Champagne-Ardenne, Reims, France (QM, ST, RJ, AS); Department of Hepatology and Liver Intensive Care, Assistance Publique-Hôpitaux de Paris, Clichy, France (MB, FD); and Department of General, Digestive and Endocrine Surgery, Robert-Debré Hospital, Université de Reims Champagne-Ardenne, Reims, France (RK).
authors.forename: Quentin
authors.initials: Q
authors.lastname: Maestraggi
authors.forename: Mohamed
authors.initials: M
authors.lastname: Bouattour
authors.forename: Ségolène
authors.initials: S
authors.lastname: Toquet
authors.forename: Roland
authors.initials: R
authors.lastname: Jaussaud
authors.forename: Reza
authors.initials: R
authors.lastname: Kianmanesh
authors.forename: François
authors.initials: F
authors.lastname: Durand
authors.forename: Amélie
authors.initials: A
authors.lastname: Servettaz
mesh_terms: D020533:Angiogenesis Inhibitors / Q000009:adverse effects* / Q000627:therapeutic use; D000068258:Bevacizumab / Q000009:adverse effects* / Q000627:therapeutic use; D001649:Bile Duct Diseases / Q000188:drug therapy* / Q000209:etiology; D006502:Budd-Chiari Syndrome / Q000139:chemically induced; D005260:Female; D006325:Heart Atria; D006331:Heart Diseases / Q000139:chemically induced; D006801:Humans; D008875:Middle Aged; D011655:Pulmonary Embolism / Q000139:chemically induced; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*; D013927:Thrombosis / Q000139:chemically induced
chemical_list: D020533:Angiogenesis Inhibitors; D000068258:Bevacizumab