Abstract
Background: We examined the severity of epistaxis in patients with hereditary haemorrhagic telangiectasia (HHT) and its relationship with the performed activities of daily living. Methodology: This cross-sectional survey included 36 patients with HHT in Japan. An anonymous questionnaire survey was conducted regarding the severity of epistaxis, the measures adopted to prevent epistaxis, and the limitations in the performed activities of daily living. The latter was assessed using a visual analogue scale (VAS). The correlation between epistaxis severity and the VAS score was analysed using Spearman’s rank correlation coefficient. Results: Of the 36 participants surveyed, 94.4% had >1 episode of epistaxis/week. The mean epistaxis severity score (ESS) was 4.3 (range, 0.9–8.4). Limitations in daily life, going out (within a day), meeting with others, eating with others, and going on overnight trips were positively correlated with the ESS. To prevent nosebleeds, 44.4% and 41.7% of the participants used medications and avoided drying their nasal cavities, respectively. Conclusions: Epistaxis impacted the daily life of patients with HHT in proportion to its severity. Nonetheless, less than half of the patients used medications or took precautions. Hence, further educational activities should be considered for medical professionals and patients.
Highlights
IntroductionHereditary haemorrhagic telangiectasia (HHT), as named by Hanes in 1909 ( known as Osler–Weber–Rendu or Osler's disease[1–4]), is a systemic disease characterised by telangiectasis and arteriovenous malformations in various organs
Hereditary haemorrhagic telangiectasia (HHT), as named by Hanes in 1909, is a systemic disease characterised by telangiectasis and arteriovenous malformations in various organs
We examined the severity of epistaxis in patients with hereditary haemorrhagic telangiectasia (HHT) and its relationship with the performed activities of daily living
Summary
Hereditary haemorrhagic telangiectasia (HHT), as named by Hanes in 1909 ( known as Osler–Weber–Rendu or Osler's disease[1–4]), is a systemic disease characterised by telangiectasis and arteriovenous malformations in various organs. In the field of otorhinolaryngology, it is well known as the cause of intractable epistaxis[5]. It is diagnosed based on the Curaçao clinical diagnostic criteria[6]. HHT is an autosomal-dominant genetic disorder caused by mutations in the ENG and ACVRL1 genes, referred to as HHT1 and HHT2, respectively[5]. Patients with severe epistaxis based on the ESS are more likely to have affected Physical Component Summary and present significantly. We examined the severity of epistaxis in patients with hereditary haemorrhagic telangiectasia (HHT) and its relationship with the performed activities of daily living
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