Medical treatment of epistaxis in hereditary hemorrhagic telangiectasia: An evidence-based review

Ashleigh A. Halderman, Matthew W. Ryan, Christopher Clark, Raj Sindwani, Douglas D. Reh, David M. Poetker, Rosangela Invernizzi, Bradley F. Marple

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder resulting in vascular dysplasia and formation of arteriovenous malformations. Recurrent epistaxis is a hallmark of the disease. An array of medical therapies are used in this patient population, but robust evidence-based recommendations regarding the medical treatment of epistaxis are lacking. This systematic review was performed to look at the current literature and make meaningful evidence-based recommendations. Methods: A search of the Ovid MEDLINE, Embase, and Cochrane databases was conducted by a research librarian. Abstracts in the English language and published in a peer-review journal were reviewed for relevance and inclusion. PRISMA guidelines were followed. Results: Eighteen studies met the inclusion criteria. In a few small studies, thalidomide was shown to consistently improve severity and frequency of epistaxis and improve hemoglobin concentrations while decreasing the need for transfusion. Tranexamic acid appeared to only impact the epistaxis severity score and not other clinical outcomes. Selective estrogen modulators (SERMs), propranolol, rose geranium oil, and N-acetylcysteine, have demonstrated promising efficacy in small trials. Conclusion: Appropriate medical therapies for epistaxis outcomes in HHT remain undefined, and there is no "gold standard." Many of the studies are small and the data reported are heterogeneous, and therefore the ability to make strong evidence-based recommendations is limited. However, many different medications appear to be promising options.

Original languageEnglish (US)
JournalInternational Forum of Allergy and Rhinology
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Hereditary Hemorrhagic Telangiectasia
Epistaxis
Geranium
Librarians
Tranexamic Acid
Therapeutics
Peer Review
Aptitude
Thalidomide
Arteriovenous Malformations
Acetylcysteine
Propranolol
MEDLINE
Blood Vessels
Estrogens
Oils
Hemoglobins
Language
Databases
Guidelines

Keywords

  • Epistaxis
  • Evidence-based medicine
  • Hemorrhagic disorders
  • Hereditary

ASJC Scopus subject areas

  • Immunology and Allergy
  • Otorhinolaryngology

Cite this

Medical treatment of epistaxis in hereditary hemorrhagic telangiectasia : An evidence-based review. / Halderman, Ashleigh A.; Ryan, Matthew W.; Clark, Christopher; Sindwani, Raj; Reh, Douglas D.; Poetker, David M.; Invernizzi, Rosangela; Marple, Bradley F.

In: International Forum of Allergy and Rhinology, 01.01.2018.

Research output: Contribution to journalArticle

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AB - Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder resulting in vascular dysplasia and formation of arteriovenous malformations. Recurrent epistaxis is a hallmark of the disease. An array of medical therapies are used in this patient population, but robust evidence-based recommendations regarding the medical treatment of epistaxis are lacking. This systematic review was performed to look at the current literature and make meaningful evidence-based recommendations. Methods: A search of the Ovid MEDLINE, Embase, and Cochrane databases was conducted by a research librarian. Abstracts in the English language and published in a peer-review journal were reviewed for relevance and inclusion. PRISMA guidelines were followed. Results: Eighteen studies met the inclusion criteria. In a few small studies, thalidomide was shown to consistently improve severity and frequency of epistaxis and improve hemoglobin concentrations while decreasing the need for transfusion. Tranexamic acid appeared to only impact the epistaxis severity score and not other clinical outcomes. Selective estrogen modulators (SERMs), propranolol, rose geranium oil, and N-acetylcysteine, have demonstrated promising efficacy in small trials. Conclusion: Appropriate medical therapies for epistaxis outcomes in HHT remain undefined, and there is no "gold standard." Many of the studies are small and the data reported are heterogeneous, and therefore the ability to make strong evidence-based recommendations is limited. However, many different medications appear to be promising options.

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