5-year experience at a single center: Retrospective analysis of 38 patients with hereditary angioedema: A descriptive study

Abstract

Objective: Hereditary angioedema (HAE) is a rare auto-somal dominant disorder characterized by recurrent angioedema attacks, without itching or urticaria. With this study, we aimed to increase the awareness of HAE by presenting the characteristics of these patients who applied to the emergency department and allergy immunology clinic. Material and Methods: A total of 38 patients, 25 (65.8%) female and 13 (34.2%) male, were included. Results: The mean age was 40.90±12.66, mean age at the onset of symptoms was 13.5 (1-56), mean age at HAE diagnosis was 24.61±13.78, and the diagnostic delay was 8.84±8.97 years. Of all cases, 18 (47.4%) were followed-up with Type I HEA and 20 (52.6%) were followed up with Type II HAE. A family history of HAE was present in 89.5% and a family history of death due to HAE was present in 31.6% of the patients. Mean age at diagnosis differed significantly between those with or without a family history of death due to HAE (18.0±7.24 vs. 27.65±15.08 years; p=0.043). The episodes were triggered by stress in 20 (52.6%) patients. The symptoms at first presentation included swelling in extremities in 18 (47.4%) patients. Conclusion: Although HAE is a rare disorder associated with variable clinical presentations complicating the diagnostic process, it may also be associated with mortality. Periodic reporting of clinical experience from centers dealing with HAE patients bears significance not only for increasing awareness among medical professionals and preventing diagnostic delays but also for improving the life quality of patients as well as for decreasing the morbidity and mortality.