The Natural History of Epistaxis in Patients with Hereditary Hemorrhagic Telangiectasia in the Norwegian population: A Cross-sectional Study

Abstract

Epistaxis is usually the first and most common symptom in hereditary hemorrhagic telangiectasia (HHT), which is known also as Rendu-Osler-Weber syndrome. The severity of HHT-associated epistaxis is highly variable and can affect the patient's quality of life. In the literature, the natural history of epistaxis in HHT patients has been described in a few countries but not from the Norwegian population. This work focused on the natural history of epistaxis in the Norwegian population in a cross-sectional study. Ninety-eight patients with three or four Curaçao criteria were included. The severity of epistaxis was graded depending on epistaxis intensity, frequency, and the amount of blood transfusion during a period of 4 weeks. The epistaxis grades were studied in association with age, gender, gene mutation, age of onset, and whether the patient had or had not been treated for epistaxis during the last 2 years. Most of the HHT patients (90%) complained of mild-to-moderate epistaxis. Seventy-seven percent of the patients started epistaxis by or before the age of 20 years. The progression of HHT-associated epistaxis with age could not be proved statistically in this study. There was no statistically significant difference in the grades of epistaxis between HHT1 and HHT2 type, neither between female and male patients. Most of the patients started epistaxis by or before the age of 20 years. There was a significant difference in the grade of epistaxis between non-ENG, non-ALK1 carrier patients, and ENG or ALK1 carrier patients. Compared with other populations, the grading of epistaxis in Norwegian patients with HHT gave generally similar results. A multicenter epidemiological study is required to get a larger study population. A common internationally accepted grading or classification system for epistaxis in HHT is highly recommended.