Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study
OBJECTIVES: 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 pu...
| Main Authors: | , , , , |
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| Format: | Article |
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American Academy of Neurology
2015
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| Online Access: | https://eprints.nottingham.ac.uk/37992/ |
| _version_ | 1848795575270506496 |
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| author | Donaldson, J.W. McKeever, Tricia M. Hall, Ian P. Hubbard, R.B. Fogarty, Andrew W. |
| author_facet | Donaldson, J.W. McKeever, Tricia M. Hall, Ian P. Hubbard, R.B. Fogarty, Andrew W. |
| author_sort | Donaldson, J.W. |
| building | Nottingham Research Data Repository |
| collection | Online Access |
| description | OBJECTIVES:
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. |
| first_indexed | 2025-11-14T19:34:16Z |
| format | Article |
| id | nottingham-37992 |
| institution | University of Nottingham Malaysia Campus |
| institution_category | Local University |
| last_indexed | 2025-11-14T19:34:16Z |
| publishDate | 2015 |
| publisher | American Academy of Neurology |
| recordtype | eprints |
| repository_type | Digital Repository |
| spelling | nottingham-379922020-05-04T17:08:57Z https://eprints.nottingham.ac.uk/37992/ Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study Donaldson, J.W. McKeever, Tricia M. Hall, Ian P. Hubbard, R.B. Fogarty, Andrew W. OBJECTIVES: 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. American Academy of Neurology 2015-05-05 Article PeerReviewed Donaldson, J.W., McKeever, Tricia M., Hall, Ian P., Hubbard, R.B. and Fogarty, Andrew W. (2015) Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study. Neurology, 84 (18). pp. 1886-1893. ISSN 1526-632X http://www.neurology.org/content/84/18/1886 doi:10.1212/WNL.0000000000001538 doi:10.1212/WNL.0000000000001538 |
| spellingShingle | Donaldson, J.W. McKeever, Tricia M. Hall, Ian P. Hubbard, R.B. Fogarty, Andrew W. Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study |
| title | Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study |
| title_full | Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study |
| title_fullStr | Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study |
| title_full_unstemmed | Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study |
| title_short | Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study |
| title_sort | complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study |
| url | https://eprints.nottingham.ac.uk/37992/ https://eprints.nottingham.ac.uk/37992/ https://eprints.nottingham.ac.uk/37992/ |