Medical treatment of epistaxis in hereditary hemorrhagic telangiectasia: an evidence‐based review

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 r...

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Bibliographic Details
Published in:International forum of allergy & rhinology 2018-06, Vol.8 (6), p.713-728
Main Authors: Halderman, Ashleigh A., Ryan, Matthew W., Clark, Christopher, Sindwani, Raj, Reh, Douglas D., Poetker, David M., Invernizzi, Rosangela, Marple, Bradley F.
Format: Article
Language:English
Subjects:
Acetylcysteine
Administration, Oral
Administration, Topical
Angiogenesis Inhibitors - therapeutic use
Clinical trials
Dysplasia
epistaxis
Epistaxis - drug therapy
Epistaxis - etiology
Estriol - administration & dosage
Estrogen Replacement Therapy - methods
Evidence-Based Medicine
Female
Hemoglobin
Hemorrhage
hemorrhagic disorders
hereditary
Hereditary diseases
Hereditary hemorrhagic telangiectasia
Humans
Male
Propranolol
Recurrence
Selective estrogen receptor modulators
Selective Estrogen Receptor Modulators - therapeutic use
Telangiectasia, Hereditary Hemorrhagic - complications
Thalidomide
Thalidomide - therapeutic use
Tranexamic Acid - therapeutic use
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Summary: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. s 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.
ISSN:2042-6976
2042-6984
DOI:10.1002/alr.22094