Effect of COVID-19 on hereditary angioedema activity and quality of life

Abstract

Background: The demonstration that severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) enters the cell via the angiotensin-converting enzyme 2 receptor has raised concerns that, in hereditary angioedema (HAE), a disease characterized by bradykinin-mediated angioedema attacks, coronavirus disease 2019 (COVID-19) may trigger angioedema attacks, increase the frequency and/or severity of attacks, or cause more severe symptoms of COVID-19. Objective: The objective was to evaluate the severity of COVID-19 in patients with HAE, the course of HAE attacks, angioedema activity, and the quality-of-life scores during COVID-19 pandemic. Methods: Patients diagnosed with HAE for at least 6 months were included in the study. The 7-day Angioedema Activity Score and the Angioedema Quality of Life (AE-QoL) Questionnaire were first completed at the onset of the pandemic between March 12 and June 1, 2020, then during SARS-CoV-2 infection, and in the third month after recovering from COVID-19. Results: Ten of 67 patients with HAE (14.9%) were diagnosed with COVID-19. The median (interquartile range) age of the 10 patients diagnosed with COVID-19 was 35.5 years (28.0‐55.0 years). Six of the 10 patients (60%) were women. During COVID-19, five of the 10 patients (50%) had no angioedema attack. Two patients with severe HAE experienced a significant increase in angioedema activity during COVID-19 compared with their basal activity scores. The remaining three patients had a similar or lower attack frequency than their basal level. Four (40%) of the 10 patients had a relative increase in their attacks during the convalescence period. There was no statistically significant difference among pre-COVID-19, during COVID-19 and post-COVID-19 periods in function, mood, fear and/or shame, nutrition, and total scores of the AE-QoL Questionnaire although the fear dimension was relatively more affected (p = 0.06). Conclusion: Although the sample size was small, analysis of our data supported that the symptoms of COVID-19 were not more severe in HAE. Also, there was no significant difference in the AE-QoL Questionnaire scores, the frequency, and severity of angioedema attacks during the course of COVID-19 in the patients with HAE.

The Interplay of COVID-19 and Hereditary Angioedema: Preventing an Acute Attack

“…When analyzing this prevalence, there have been many concerns about an escalation in the severity of HAE attacks or even worsening the effects of the COVID-19 virus. In a study assessing the quality of life points for those with HAE within six months of diagnosis, It was found that half of their cohort experienced worse angioedematous attacks during their COVID-19 infection, while the other portion of the cohort reported insignificant findings that were in line with the control population [2].…”

“…In a study of 67 patients with HAE, only 10 patients (14.9%) were diagnosed with COVID-19. While the patients were infected with COVID-19, five out of 10 patients experienced HAE attacks [2]. The differentiating factor of patients with HAE is the level of C1 inhibitors present in the patient [3].…”

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

“…Initial data from Brazil, France, and Turkey showed no significant increase in HAE-1/2 activity during or after COVID-19, although some patients who were only using on-demand therapy did report increased HAE activity. 380 , 381 , 382 , 383 A manuscript, in press, using the database of the HAE-A demonstrated no evidence that HAE patients were at greater risk for infection or for serious infection, nor adverse effects to the vaccine. In a recent survey from the Netherlands, following 111 COVID-19 vaccine doses administered, 11 attacks were reported, 6 arose more than 48 h after vaccination.…”

Mechanisms, diagnostics and therapies for thromboinflammation

“…45 Conversely, COVID-19 disease does not appear to be aggravated in HAE patients. 45,46 A potential role for contact activation (targeting therapy) diagnostics in COVID-19 disease?…”

“…Besides organ damage, these syndromes are clinically characterized by a low platelet count (thrombocytopenia), and microangiopathic haemolytic anemia. 45 Although the incidence of TTP is relatively low (50-100 patients in the Netherlands, 46 the potential lethality of a an untreated TTP attack calls for rapid differentiation from other types of TMA. Measuring ADAMTS13 activity is key to this differential diagnosis: other types of TMA might have reduced ADAMTS13 activity whereas a severe deficiency (<10%) of ADAMTS13 activity is highly specific for TTP.…”