Validation of epistaxis severity score for hereditary hemorrhagic telangiectasia in Japan. Issue 3 (June 2022)

• Record Type: Journal Article
• Authors: Hayama, Masaki; Maeda, Yohei; Shikina, Takashi; Tatehara, Shun; Inokuchi, Go; Hoag, Jeffrey B; Sato, Tomoharu; Obata, Sho; Nakatani, Ayaka; Amano, Yuta; Hida, Eisuke; Nibu, Ken-Ichi; Inohara, Hidenori
• Is Part Of: Auris nasus larynx. Volume 49:Issue 3(2022)
• Page Start: 415
• Page End: 420
• Publication Date: 2022-06
• Subjects: Hereditary hemorrhagic telangiectasia -- Quality of life -- Epistaxis -- Severity of illness index -- Surveys and questionnaires; Otolaryngology -- Periodicals; Electronic journals; 616
• DOI: 10.1016/j.anl.2021.11.005
• Languages: English
• ISSNs: 0385-8146

Abstract

Abstract: Objective: This study aimed to assess the health-related QoL (HR-QoL) of patients with hereditary hemorrhagic telangiectasia (HHT), with emphasis on the role/social aspects, and validate the Japanese version of the epistaxis severity score (ESS) in these patients. Methods: The Japanese version of the ESS was created through forward and reverse translation, and consultation with the original author. A validation analysis was performed by comparing ESS severity with the invasiveness of previous treatments for epistaxis and assessing the correlation between the ESS and HR-QoL. Medical history forms, ESS questionnaires, and the Medical Outcomes Study Short Form 36 (SF-36) were distributed to participants with HHT in August 2020. The relation between the ESS and summary scores of SF-36 was assessed by performing analysis of variance and Spearman's correlation. Results: In total, 73 participants were included in this study. The average ESS was 5.02; there were mild (32.9%), moderate (45.2%), and severe (21.9%) epistaxis groups. Patients with higher ESS received a significantly more invasive treatment (Fisher's exact test, p < 0.05). The ESS was also negatively correlated with the physical component score (PCS) ( r = -0.489, p < 0.001). Comorbid liver and gastrointestinal arteriovenous malformations significantly reduced the PCS ( p < 0.05). Multiple regression analysis revealed that the ESS was a significant variable ( p < 0.01). The role/social component score was significantly lower in the severe ESS group than in the mild or moderate group. Conclusion: The Japanese version of the ESS was considered valid and may be useful as an outcome measure of future HHT-associated epistaxis trials in Japan.