How Angioedema Quality of Life Questionnaire Can Help Physicians in Treating C1-Inhibitor Deficiency Patients?

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.

The Missing Link: A Case of Severe Adverse Reaction to Histamine in Food and Beverages

“…HAE triggers include alcohol, infection, mental stress, surgical procedures, and medication [ 18 ]. Quality of life is strongly impacted [ 19 ]. Prodromal symptoms include fatigue, gastrointestinal flu-like symptoms, and erythema marginatum.…”

The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update

“…Patients with HAE should monitor their disease activity, impact, and control, and this is especially important in patients who use long-term prophylactic treatment (Recommendation 19). 184 , 246 , 277 , 278 , 279 Validated patient-reported outcome measures such as the angioedema activity score (AAS), 192 , 280 the hereditary angioedema activity score (HAE-AS), 186 the angioedema quality of life questionnaire (AE-QoL), 191 , 193 , 281 the hereditary angioedema quality of life questionnaire (HAE-QoL), 187 , 188 and the angioedema control test (AECT) 189 , 190 are available in a wide range of languages and should be used for this purpose 277 , 282 The aims of effective HAE treatment, ie, the absence of attacks, normalization of QoL, and complete control, are best achieved when assessed by appropriate tools.…”

Long term follow-up of complement parameters to improve the management of acquired angioedema due to C1-inhibitor deficiency

“…Both are characterized by recurrent subcutaneous and/or submucosal angioedema attacks. Both the hereditary and the acquired form can occur due to C1-inhibitor (C1–INH) deficiency (C1–INH-HAE, C1–INH-AAE) [ 1 , 2 , 3 , 4 , 5 ]. C1–INH-AAE is a rare disorder, with an approximate incidence rate of 1:500,000, the family history is negative, and the angioedema symptoms occur over the age of 40 years [ 2 , 6 , 7 , 8 , 9 , 10 , 11 ].…”