title: Complications and mortality in hereditary hemorrhagic telangiectasia: A population-based study.
abstract: OBJECTIVE
  Studies report that the risks of significant neurologic complications (including stroke, cerebral abscess, and migraine) and hemorrhagic sequelae are high in patients with hereditary hemorrhagic telangiectasia (HHT), and that life expectancy in this cohort is reduced. However, most published cohorts derive from specialist centers, which may be susceptible to bias.
  
  
  METHODS
  We used a population-based approach to estimate the risks of developing neurologic and hemorrhagic complications of HHT, the association of a diagnosis of HHT with common cardiovascular and malignant comorbidities, and also long-term survival of those with the disease.
  
  
  RESULTS
  From a UK primary care database of 3.5 million patients (The Health Improvement Network), we identified 675 cases with a diagnosis of HHT and compared them with 6,696 controls matched by age, sex, and primary care practice. Risks of stroke (odds ratio [OR] 1.8, 95% confidence interval [CI] 1.2-2.6), cerebral abscess (OR 30.0, CI 3.1-288), and migraine (OR 1.7, CI 1.3-2.2) were elevated over controls. Bleeding complications including epistaxis (OR 11.6, CI 9.1-14.7) and gastrointestinal hemorrhage (OR 6.1, CI 2.8-13.4) were more common in cases with HHT. Survival of cases with HHT was poorer than controls with a hazard ratio for death of 2.0 (CI 1.6-2.6) and a median age at death 3 years younger.
  
  
  CONCLUSIONS
  Patients with HHT are at substantially increased risk of serious neurologic and hemorrhagic complications of the disease. Because a diagnosis of HHT is associated with a significantly poorer survival compared with those who have no disease, evaluation of new strategies to improve clinical management is required.
authors: From the Divisions of Epidemiology and Public Health (J.W.D., T.M.M., R.B.H., A.W.F.) and Therapeutics and Molecular Medicine (I.P.H.), University of Nottingham, UK. james.donaldson@nottingham.ac.uk.
authors: James W
authors: JW
authors: Donaldson
authors: From the Divisions of Epidemiology and Public Health (J.W.D., T.M.M., R.B.H., A.W.F.) and Therapeutics and Molecular Medicine (I.P.H.), University of Nottingham, UK.
authors: Tricia M
authors: TM
authors: McKeever
authors: From the Divisions of Epidemiology and Public Health (J.W.D., T.M.M., R.B.H., A.W.F.) and Therapeutics and Molecular Medicine (I.P.H.), University of Nottingham, UK.
authors: Ian P
authors: IP
authors: Hall
authors: From the Divisions of Epidemiology and Public Health (J.W.D., T.M.M., R.B.H., A.W.F.) and Therapeutics and Molecular Medicine (I.P.H.), University of Nottingham, UK.
authors: Richard B
authors: RB
authors: Hubbard
authors: From the Divisions of Epidemiology and Public Health (J.W.D., T.M.M., R.B.H., A.W.F.) and Therapeutics and Molecular Medicine (I.P.H.), University of Nottingham, UK.
authors: Andrew W
authors: AW
authors: Fogarty
mesh_terms: D000293:Adolescent; D000328:Adult; D000740:Anemia / Q000209:etiology; D001922:Brain Abscess / Q000209:etiology*; D016022:Case-Control Studies; D002543:Cerebral Hemorrhage / Q000209:etiology; D016208:Databases, Factual; D004844:Epistaxis / Q000209:etiology*; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D008881:Migraine Disorders / Q000209:etiology*; D016017:Odds Ratio; D016016:Proportional Hazards Models; D020521:Stroke / Q000209:etiology*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000401:mortality; D006113:United Kingdom; D055815:Young Adult