Altered quality of life in Rendu-Osler-Weber disease related to recurrent epistaxis

Objectives: Development and validation of an epistaxis-specific quality-of-life questionnaire (EQQoL) to evaluate the impact on quality of life of epistaxis, during hereditary hemorrhagic telangiectasia (HHT). Study design: Prospective clinical study using QoL instruments administered twice in HHT patients. Patients and methods: In total, 109 patients who had epistaxis and a clinical diagnosis of HI-IT according to Curacao criteria were included. Invoice of the questionnaire in 2004 and 2006 included SF-36, Jenkins' sleep scale and the new epistaxis-specific13-item EQQoL. Results: EQQoL uptake rate was 98%, mean score 581100 +/- 27, and Cronbach alpha 0.96. EQQoL was sensitive to change with a strong correlation with the course of epistaxis. Factorial analysis showed that EQQoL was clearly distinct from SF-36 and Jenkins sleep scales. In stepwise multivariate ordinal logistic regression, frequency and duration of epistaxis were both associated with lower EQQoL. Conversely, visceral involvement and comorbidity had independent impact on SF-36 scores, but not on EQQoL. Conclusions: This new epistaxis-specific EQQoL questionnaire provides complementary information on the impact of HI-IT on patients' quality of life relative to the SF-36 generic questionnaire. After international validation, the EQQoL might prove a useful tool for treatment evaluation.