Influence of hormonal changes in patients with hereditary hemorrhagic telangiectasia

Abstract

Abstract Background:Hereditary Hemorrhagic Telangiectasia (HHT) is a rare and systemic vascular disorder which is characterized by recurrent epistaxis and mucocutaneous telangiectasia. The role of hormones in reducing epistaxis in these patients is assumed but so far clear evidence is missing. An electronic questionnaire in English and German was distributed which included sections about the general medical history of HHT and the influence of hormonal changes on the disease symptoms. Results:Of the 477 respondents with HHT 326 were female, i.e. 68%. The mean age of female patients was 54 years (standard deviation: ± 11.5 years, range [28, 83]). With aging, females suffered from more epistaxis compared to male patients. Out of the 228 female patients who stated that they had ever taken hormones, 73% have been taking hormones for contraception, 57% for hyper-/dysmenorrhea and 26% for menopausal discomfort. Under hormone intake 41% of the women with HHT stated an improvement of their nosebleeds, and 24% that their telangiectasia decreased. Patients who took estrogen and progestin combinations reported less epistaxis. Most female patients reported that their epistaxis increased during puberty (41%) and during pregnancy (39%), mainly in the second trimester. Conclusions:In HHT, variation in levels of hormone concentrations seemed to contribute to symptom severity. A tentative off-label use of hormonal products might be discussed with women. Clinicaltrials.gov: NCT02690246