Objective: To assess and differentiate the health-related quality of life (HR-QoL) in patients with hereditary hemorrhagic telangiectasia (HHT).
Study design and setting: A prospective, open, cross-sectional questionnaire-based study (including the Short Form-36 Health Survey [SF-36]) performed by a tertiary care center.
Results: A total of 77 patients (36 females) were included. Except for one domain (bodily pain), the scores for all scales of the SF-36 were significantly reduced in comparison with normative data. The duration of epistaxis, the presence of hepatic involvement and gastrointestinal bleeding, and the number of visible telangiectases correlated with lower scores on several scales of the SF-36. Unexpectedly, the frequency of epistaxis did not correlate with any scale.
Conclusions: The duration of epistaxis, liver involvement, gastrointestinal bleeding, and the number of visible telangiectases have a major influence on the HR-QoL in HHT whereby the frequency of epistaxis seems to play a minor role.
Significance: The data presented have an impact on therapeutic decisions, medical expert opinions, and research funding.