Department of Allergy, Chinese Academy of Medical Sciences & Peking Union Medical College Hospital, Beijing 100730,
School of Clinical Medicine, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100005,
Department of Epidemiology and Biostatistics, Institute of Basic Medical Sciences Chinese Academy of Medical Sciences, School of Basic Medicine, Peking Union Medical College, Beijing 100005,
Center of Environmental and Health Sciences, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100005,
Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China
Background: Hereditary angioedema (HAE) is a rare, inherited disorder characterized by unpredictable and recurrent cutaneous and mucosal oedema. Objectives: This study aimed to identify the current status of HAE management in China. Materials and Methods: An internet-based survey was sent to 129 patients with type 1 and 2 HAE diagnosed between 1983 and 2017; a total of 107 patients provided complete disease management information. Results: The survey response rate was 82.9% (107 responders). Ten patients reported 18 fresh frozen plasma (FFP) transfusions for the main purpose of treating lethal laryngeal oedema. Two patients reported adverse events. Eighty-nine (83.2%) patients had taken danazol for long-term prophylaxis, and 74 (69.2%) were on long-term danazol prophylaxis. Among patients on long-term prophylaxis, 56 (75.7%) patients reported a decrease in their annual attack frequency after taking danazol. Twenty-five (33.8%) patients had an attack frequency of no more than once per year. The most common side effects were menstrual disorders, weight gain, osteoarticular pain, acne, and sebaceous hypersecretion. Patients with higher education levels, positive family histories, and laryngeal oedema attacks before medication tended to show better adherence. Patients maintaining high or medium adherence showed better control of laryngeal and gastrointestinal involvement than patients with low adherence. Four (3.7%) patients reported current or past use of tranexamic acid. Conclusion: Attenuated androgen and FFP transfusions remain the mainstay in China, where specifically targeted drugs are currently lacking. Maintaining good medication adherence should be recommended to achieve improved disease control.