Abstract

Epistaxis is the most prevalent clinical symptom in Hereditary Haemorrhagic Telangiectasia (HHT), causing anaemia and decreasing the quality of life (QOL). Since 2013, in Hospital Universitario Fundación Alcorcón, more than 150 HHT patients have been treated by nose sclerotherapy on demand. This study shows the results of 105 patients treated with sclerotherapy between 2017 and 2019. HHT-ESS (epistaxis severity score) was used to measure the severity and frequency of epistaxis. QOL was determined before and after treatment by EuroQol-5D (EQ-5D) and the visual analogue scale (VAS) on the health condition. According to HHT-ESS before treatment, 22 patients presented mild, 35 moderate, and 47 severe epistaxes. Sclerotherapy significantly decreased the frequency and severity of epistaxis, with a significant drop of HHT-ESS in 4.6 points, from 6.23 ± 2.3 to 1.64 ± 1.6. Furthermore, the QOL significantly improved, the EQ-5D scale raised from 0.7 ± 0.26 pre- to 0.92 ± 0.16 post-treatment (p < 0.05). Additionally, VAS mean value showed a significant increase from 4.38 ± 2.4 to 8.35 ± 1.2. The QOL improvement was correlated with the ESS decrease. In conclusion, this study shows that on-demand sclerotherapy at the office significantly reduces HHT epistaxis as well as improved the patients’ QOL.

Highlights

  • quality of life (QOL) was measured by two scales—EQ-5D and visual analogue scale (VAS)

  • The results presented in this study s demonstrate that on-demand sclerotherapy significant

  • The results presented in this study s demonstrate that on-demand sclerotherapy with polidocanol is a highly effective therapy, in reducing the severity of bleedings in the Haemorrhagic Telangiectasia (HHT) population according to the HHT-ESS score and in improving their QOL

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Summary

Introduction

Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Hereditary haemorrhagic telangiectasia (HHT), known as Rendu–Osler–Weber disease, is an autosomal dominant multisystemic vascular disorder with incomplete penetrance. The estimated prevalence ranges from 1:5000 to 1:8000 [1], and it is considered a rare disease. HHT affects several organs and is the cause of a wide variety of clinical manifestations. The diagnosis for HHT is achieved by the Curaçao criteria [2]: epistaxis, mucocutaneous telangiectases, first-degree family inheritance, and visceral arteriovenous malformations (AVM). The presence of three of these four criteria results in a conclusive

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