The Natural Course of Hereditary Angioedema in a Chinese Cohort

Abstract Background Hereditary angioedema (HAE) is a rare disease with potential life-threatening risks. Until now, few studies have focused on the natural course of HAE, which is essential for practitioners and patients to predict disease progression and avoid possible risk factors. Objectives Describe the natural course of HAE and explore possible risk factors, providing new clues for guiding clinical prevention and treatment. Methods A web-based survey was conducted in 103 Chinese patients with type 1 HAE. Disease progression under therapy-free conditions was collected at different age stages. The demographic characteristics, lifestyles, disease severity and family history were also provided by each participant. Results Among 103 patients, 14 (13.6%) had their first HAE attack before ten years old and 51 (49.5%) between 10 and 19. The disease worsened in 83.3% of the patients in their twenties. The proportion of patients with symptoms alleviated increased after the age of 30 years old, but the disease maintained relatively severe in most cases before 50. The participants also reported 233 members shared similar symptoms of angioedema in their family and 30 had died of laryngeal edema with the median death age of 46 years old. The disease severity was not observed to be affected significantly by gender, BMI, alcohol or smoking. Conclusions We summarized HAE progression patterns in therapy-free conditions, showing the natural course of disease development along with aging. Long-term prophylaxis and symptomatic treatment are recommended for all HAE patients, especially young and middle-aged and might be adjusted depending on the disease progression.