gms | German Medical Science

Background: Only few data exists about health-related quality of life (HR-QoL) in HHT. This data might be helpful for therapeutical decisions and the choice of research priorities.

Methods: The Short Form-36 Health Survey (SF-36) was administered to 77 HHT patients and scores were compared to a normal cohort. A multitude of other variables were additionally obtained from the patients.

Results: HHT patients answered to the open questions on what is responsible for impediments in private or occupational life and for psychic strain by naming epistaxis most frequently. HHT patients had significantly lower scores for all multi-item scales of the SF-36 except bodily pain, the highest standardized difference was found for role emotional. The scores for most scales were significantly lower among females. In bivariate analyses longer epistaxis episodes were related to lower physical functioning (p = 0.002, physical role limitations (p = 0.023) and the physical component summary (p = 0.012). Unexpectedly, no significant correlation could be found between epistaxis frequency and any scale. Pulmonary and cerebral vascular malformations correlated significantly (p < 0.05) with one scale each, gastrointestinal involvement and number of telangiectases with several scales. The visible number of telangiectases, subjective dyspnea and number of transfusions were related to lower scores in several scales (p < 0.05). Liver involvement was correlated with lower scores in all scales (p < 0.05). Conclusions: HR-QoL is reduced in HHT patients. Duration of epistaxis episodes, hepatic and gastrointestinal involvement, and the number of telangiectases seem to be of major importance for HR-QoL in HHT.