title: Intravenous bevacizumab for complications of hereditary hemorrhagic telangiectasia: a review of the literature.
abstract: BACKGROUND
  Hereditary hemorrhagic telangiectasia (HHT) is a multisystem disease that is marked by mutations regulating vasculature formation. Epistaxis is the most commonly reported symptom, but gastrointestinal bleeding, anemia, hepatic issues, and pulmonary disease are also common. There is a growing body of evidence in the literature concerning using the monoclonal antibody against vascular endothelial growth factor (VEGF), bevacizumab, in patients with HHT. This treatment is gaining support for managing HHT because it directly inhibits the VEGF proteins that can be elevated as a result of the HHT mutations. We reviewed the current literature on the outcomes from intravenous bevacizumab treatment for HHT with a focus on epistaxis outcomes.
  
  
  METHODS
  A systematic review of the literature was performed using Ovid MEDLINE, Scopus, and Cochrane databases. English citations, both national and international, were reviewed and filtered for relevance.
  
  
  RESULTS
  Eighteen studies were included in this review. The majority of citations were case reports. All studies reported improvements. Specifically, 14 reported improvements in epistaxis, and 11 reported hemoglobin improvement following intravenous (IV) bevacizumab. Lack of uniformity in data presentation prevented a meta-analysis.
  
  
  CONCLUSIONS
  This is the first systematic review analyzing the data involving HHT patients treated with bevacizumab. The results show that patients treated with bevacizumab have global improvements as well as specific improvements in hemoglobin levels. Although all of the studies reported improvements, there are several limitations, including inconsistencies in outcome reporting. A large, randomized, controlled study is needed to further investigate hemorrhage and epistaxis outcomes in HHT patients treated with intravenous bevacizumab.
authors.affiliation: Department of Otolaryngology and Communication Sciences, Medical College of Wisconsin, Milwaukee, WI.|Department of Surgery, Division of Otolaryngology, Zablocki VA Medical Center, Milwaukee, WI.
authors.forename: Natalia P
authors.initials: NP
authors.lastname: Arizmendez
authors.affiliation: Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Luke
authors.initials: L
authors.lastname: Rudmik
authors.affiliation: Department of Otolaryngology and Communication Sciences, Medical College of Wisconsin, Milwaukee, WI.|Department of Surgery, Division of Otolaryngology, Zablocki VA Medical Center, Milwaukee, WI.
authors.forename: David M
authors.initials: DM
authors.lastname: Poetker
mesh_terms: D020533:Angiogenesis Inhibitors / Q000627:therapeutic use*; D000818:Animals; D000068258:Bevacizumab / Q000627:therapeutic use*; D006801:Humans; D007167:Immunotherapy; D007275:Injections, Intravenous; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000276:immunology / Q000628:therapy*; D042461:Vascular Endothelial Growth Factor A / Q000276:immunology
chemical_list: D020533:Angiogenesis Inhibitors; D042461:Vascular Endothelial Growth Factor A; D000068258:Bevacizumab
keywords: VEGF; anemia; bevacizumab; epistaxis; hemorrhage; hereditary hemorrhagic telangiectasia