Evaluation of quality of life in Danish patients with Hereditary Haemorrhagic Telangiectasia and validation of Epistaxis Severity Score

Objective This study aimed to assess the quality of life in patients with Hereditary Haemorrhagic Telangiectasia (HHT) and to validate the Danish version of the Epistaxis Severity Score (ESS). Methods The Danish version of the ESS was created through forward backward translation. The study was conducted through The Danish HHT Database, which includes patients from the national HHT center. Participants completed the Danish ESS, Short Form 36 (SF-36), and Visual Analog Scale (VAS) questionnaires, and filled out a two-week nosebleed diary documenting bleeding time. Haemoglobin and ferritin were obtained from routine clinical follow-up. ESS was validated by correlating ESS with VAS, SF-36, and bleeding time. Quality of life was assessed using VAS, SF-36, and bleeding time. Results A total of 124 patients were included. Mean participant age was 53 years, and 65% were female. Average ESS was 3.37 distributed in a mild (62.9%), moderate (35.5%), and severe (1.6%) group. The scatter plot indicated a positive correlation between ESS and bleeding time among participants with heavier bleeding. There was a negative correlation between ESS and the SF-36 scale ”General Health” subscale (−4.14 (−6.01; −2.27), p < 0.001). All SF-36 subscales except ”bodily pain” were found to correlate to ESS. Age was found to be the only predictor of ESS, although the effect size was small (0.025). No associations were found between ESS and gender, HHT type, or anticoagulation therapy. Conclusion The Danish ESS demonstrated good construct validity and inverse correlations with multiple SF-36 domains, confirming its utility for assessing epistaxis severity and quality of life among Danish HHT patients. Although a valuable score, ESS may not represent the full clinical picture of epistaxis severity and should be interpreted alongside other assessment tools.