Background Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder with multiorgan manifestations, necessitating a multidisciplinary approach. Epistaxis is a common manifestation leading to significant morbidity. Current treatments are variable in efficacy, and adequate control of epistaxis remains a significant challenge. Objective To describe the cohort of patients treated at a dedicated multidisciplinary HHT clinic and to evaluate the efficacy of an HHT-related epistaxis treatment protocol at a multidisciplinary HHT clinic. Methods This study included both retrospective and prospective cohorts of adult patients (≥ 18 years) with confirmed HHT attending a tertiary multidisciplinary clinic. The retrospective cohort comprised patients managed prior to the establishment of the treatment protocol and contributed to baseline demographic and disease characteristics only. The prospective cohort of patients, recruited from September 2022 to September 2024, was managed using a tiered protocol progressing from topical treatment to surgical intervention to systemic therapy. Epistaxis severity score (ESS) was recorded longitudinally. Treatment efficacy was assessed by comparing ESS at baseline, pre-intervention, and post-intervention time points. Results A total of 62 patients, including 31 retrospective and 31 prospective patients, were identified with a mean age of 52.5 ± 17.4 years and female predominance of 62.9%. Baseline ESS scores were highest in patients in the “Bevacizumab” group, followed by those in the “Surgical treatment” group, and lowest in patients in the “Topical treatment only” cohort ( P = .001). Within the prospective cohort, significant improvements in ESS were observed following surgery (ΔESS = 4.31, P = .006) and bevacizumab (ΔESS = 4.74, P = .005). Nearly half (48.4%) of patients in this clinic achieved satisfactory control with conservative measures alone. Conclusion A structured, multidisciplinary protocol enables effective management of HHT-related epistaxis, matching treatment intensity to disease severity to achieve satisfactory symptom control for patients.
Peiris et al. (Fri,) studied this question.