The natural course of hereditary angioedema in a Chinese cohort

• Main Authors: Yang Cao, Shuang Liu, Yuxiang Zhi
• Format: Article
• Language: English
• Published: BMC 2020-09-01
• Series: Orphanet Journal of Rare Diseases
• Subjects: Hereditary angioedema (HAE); Natural course; Risk factors
• Online Access: http://link.springer.com/article/10.1186/s13023-020-01526-1

Abstract Background Hereditary angioedema (HAE) is a rare disease with potential life-threatening risks. To study the natural course of HAE under therapy-free conditions throughout patient life is essential for practitioners and patients to avoid possible risk factors and guide treatment. 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 at different age stages was provided by each participant. The data for exploring the natural course of HAE composed of two parts: one came from the participants who had never adopted any prophylactic drug for HAE; the other was from the patients with a history of medication, but only the periods before they got confirmed diagnosis and received medications were analyzed. The demographic characteristics, lifestyles, disease severity, and family history were also collected. Results Among 103 patients, 14 (13.6%) had their first HAE attack before 10 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 under therapy-free conditions, showing the natural course of HAE 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.