Epidemiological, clinical and endoscopic features of epistaxis severity and quality of life in Hereditary haemorrhagic telangiectasia: a cross-sectional study

Abstract

Background: Epistaxis is the main complaint in patients with Hereditary haemorrhagic telangiectasia (HHT). Even though the role of epistaxis in affecting the quality of life (QoL) is well-known, little is known about epidemiological and clinical factors contributing to epistaxis severity and QoL. Methodology: This is a cross-sectional study, including adult patients with HHT with epistaxis. All patients underwent an otolaryngological evaluation with nasal endoscopy. Epistaxis severity was graded using the FID score, and QoL was evaluated with the Short-Form Health Survey (SF-36). Descriptive statistics were produced for demographic characteristics; the Shapiro-Wilk test was used to test the normal distribution of quantitative variables. Correlation between the quantitative variables was evaluated with Pearson’s correlation coefficient. Both univariate and multivariate linear regression models were fitted to find associations between demographic or clinical factors and the FID score or SF-36. Results: A total of 234 patients with HHT were included in the study. The univariate analysis highlighted the association between high blood pressure, septal perforation, nocturnal epistaxis, surgery, blood transfusion, hormonal therapy and both FID score and QoL. Sex, allergic rhinitis and nasal polyposis were neither related to epistaxis severity nor perceived health. Conclusions: Epistaxis severity and QoL in patients with HHT are influenced by several clinical factors both dependent and independent from HHT. Some of the results are consistent with those already published, but for the first time, we extended the analysis to different clinical parameters, such as endoscopic findings, never assessed before.

Silicone septal splint for recurrent epistaxis in HHT patients: experience of a national referral centre

“…Epistaxis is the most commonly reported symptom in HHT patients. The recurrence and severity of nosebleeds lead to repeated and frequent medical interventions and often translate into poor quality of life for patients with HHT 7-11 .…”

“…As far as patients with HHT are concerned, the presence of septal perforation has been proven to be an important risk factor in worsening the severity of epistaxis 7 .…”

“…In about 80% of cases nosebleeds are the first clinical symptom of the disease and appear before the age of 20 in more than 50% of patients 5 , 6 . Epistaxis varies in intensity, frequency and duration but its severity is often moderate to serious and significantly affects patients’ quality of life 7-11 .…”

“…Moreover, patients with recurrent nosebleeds who have already undergone multiple surgical procedures and have developed complications of surgery, such as septal perforation, are known to be a much more difficult court to treat 7 .…”

Hereditary Hemorrhagic Telangiectasia in Pediatric Age: Focus on Genetics and Diagnosis

“…Gonzalez et al [ 27 ] provided a good description of the epistaxis features in HHT children and attempted to define a severity score. The issue of the definition of a severity score has been addressed several times [ 28 , 29 , 30 ], as it may be very relevant to evaluating the efficacy of therapies, both in adults and children. As the severity of nosebleeds is usually mild in children, their treatment mainly includes humidification, topical moisturizing, and hemostatic products, while invasive procedures or drugs with possible adverse effects (ablation therapy, septodermoplasty, nasal closure, systemic antifibrinolytic therapy, antiangiogenic agents) are prescribed to adults only.…”