How physicians approach hereditary angioedema: A single center study

Abstract

Background: Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by C1-inhibitor deficiency. It is characterized by recurrent attacks of cutaneous and upper respiratory tract swelling, and abdominal pain due to mucosal edema. Early detection and treatment prevent unnecessary interventions, improves quality of life, and prevents potentially fatal attacks. Objective: The present study aims to investigate physicians level of knowledge and awareness regarding HAE. Methods: A questionnaire about HAE was applied to 393 physicians from a university hospital. Participants were requested to choose one or several answers to multiple-choice questions. Results: Seven and three tenths percent of study participants stated to have never heard of HAE. Twenty-seven physicians (7.4%) chose the exact correct answers regarding diagnostic tests, and 2 (0.8%) chose the exact correct answers regarding emergency management. A composite of internists, pediatrists and emergency medicine specialists had a significantly higher mean score than other physicians ( p = 0.047). Physicians from internal medical sciences scored significantly higher than physicians from surgical medical sciences ( p = 0.022). Conclusion: The present study reveals that physician awareness about HAE is low, and physicians misdiagnose HAE attacks as histaminergic angioedema attacks, and therefore provide ineffective treatment. Although HAE is a rare disease, physician awareness must be increased, because early diagnosis and effective treatment are vital for the patients.