Abstract
The Angioedema Quality of Life Questionnaire (AE-QoL) is an angioedema (AE)-specific validated questionnaire, which surveys the quality of life of diagnosed patients. The questionnaire has been used in multiple clinical trials. Our aim was to investigate how the questionnaire can assist physicians in the everyday practice of following up and managing C1-inhibitor deficiency patients. In a prospective trial conducted in our center between 2016 and 2018, 125 hereditary angioedema and 10 diagnosed with acquired angioedema completed an AE-QoL during their annual follow-up visit. Laboratory indices (i.e., complement levels) were obtained for each patient. Statistical analysis comparing clinical data with QoL parameters was performed. Results of the analysis show that AE-QoL total score and number of AE attacks per year correlated well (r = 0.47; p < 0.0001). Women reached higher AE-QoL total score values than men, over a 3-year period (p = 0.0014). The highest AE-QoL total scores were reached by the 41–60-year age group; we obtained a similar result, when analyzing the four domains. No correlation was found between the AE-QoL total score and complement parameters. Patients with a negative correlation between AE-QoL total score and number of AE attacks had a positive correlation with psychologic attributes like fatigue/mood and fears/shame domains. Patients that acquired HAE showed a significant correlation between the annual number of AE attacks and the AE-QoL total scores (r = 0.46; p < 0.0001). The study establishes the use of AE-QoL as a clinical tool for follow-up which can help in the complex assessment of both hereditary and acquired HAE patients, and help to develop better therapeutic strategies.
Highlights
Angioedema (AE) with C1-inhibitor deficiency (C1-INHAE) belongs to the group of bradykinin (BK)-mediated AEs
C1-INH deficiency patients treated in the Hungarian Angioedema Center of Reference and Excellence (HACRE) had a follow-up visit at least once a year, at which we recorded the number of AE attacks that occurred in the past year; the body location of the AE attack, based on the data recorded in patient diaries; discharge reports; and other medical records
We found a smaller group of patients that show an inverse correlation between the Angioedema Quality of Life Questionnaire (AE-QoL) total score and number of AE attacks: part of this group has a worse QoL despite a low number of AE attacks, while others have many attacks, but their QoL were considered to be good
Summary
Angioedema (AE) with C1-inhibitor deficiency (C1-INHAE) belongs to the group of bradykinin (BK)-mediated AEs. These AEs are rare, potentially life-threatening, and characterized by recurrent angioedema episodes [1, 2] They can be further divided into hereditary and acquired AEs. Hereditary AE with C1-INH deficiency (C1-INH-HAE) is caused by mutations in the SERPING1 gene, with an autosomal dominant inheritance pattern [3, 4]. Acquired AE with C1-INH deficiency (C1-INH-AAE) is mostly developed due to an underlying disease (mostly lymphomas) where a consumption of the C1-INH protein and/ or formation of anti-C1-INH autoantibodies cause decreased C1-INH quantity and function [5]. Clinical manifestations of C1-INH-AE might be misdiagnosed, resulting in a diagnosis delay of years, or even decades [6, 7]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
