ABSTRACT
Background: Hereditary (HAE-C1- INH) and acquired (AAE C1-INH) angioedema with C1 inhibitor (C1 INH) deficiency are rare but potentially life-threatening conditions associated with bradykinin overproduction and recurrent episodes of angioedema without wheals. Increased susceptibility to infections or infection-associated fatal outcomes has not been previously described in this condition. However, in 2020 novel theories suggesting bradykinin as a potential mediator involved in lung injury in COVID-19 disease have been proposed. Therefore, a more severe course of COVID-19 infection in C1 INH deficient should be considered. Method(s): In September 2021, we performed a retrospective analysis of COVID-19 disease courses in HAE C1-INH and AAE C1-INH patients from Czech referral centers for the treatment of hereditary angioedema with C1 INH deficiency. Collected data involved basic demography, comorbidities, previous immunosuppressive treatment, COVID-19 symptoms and treatment, HAE symptoms during infection. Result(s): We identified 17 patients (10 females, 7 males) with C1 INH deficiency with COVID-19 positivity from March 2020 until September 2021 with median age of 45 years (10-80 years). Our cohort consisted of 16 HAE C1-INH patients (94%, HAE-1 15/ 16 -94%, HAE-2 1/16 -6%) and 1 AAE C1-INH patient (6%). Only 8 (47%) of the patients were receiving HAE prophylaxis. Most common comorbidity was obesity (4/17, 24%) followed by autoimmune disease (3/17, 18%), hypertension (3/17, 18%), immunodeficiency (3/ 17, 18%), prior immunosuppressive treatment (3/ 17, 18%) and malignancy (2/17, 12%). COVID-19 infection was asymptomatic in 3 of them (18%). Symptomatic patients reported most commonly fever (10/14, 67%), anosmia and ageusia (8/14, 53%) and headache (3/14, 20%). Only 2 symptomatic patients (14%) had pneumonia treated with antibiotics. None of our patients were treated with monoclonal antibodies or referred to the hospital. All the patients recovered. Two patients reported long-lasting symptoms more than 3 months after infection. Five patients (29%) experienced HAE attacks and in two of them, increased attack frequency lasted several weeks after recovery. Conclusion(s): According to our findings, we do not assume C1 INH deficiency to be a risk factor for a severe course of COVID-19 disease. However, as other infections, COVID-19 might trigger angioedema attacks and may cause increased attack frequency after recovery.
ABSTRACT
It has been a year since the first person on Earth became infected with a new type of coronavirus SARS-CoV-2, causing infectious acute respiratory disease COVID-19 with relatively high morbidity and mor-tality. The most endangered population by coronavirus SARS-CoV-2 are healthcare professionals, the elderly and people with associated comorbidities. Due to the fast community spread, governments of different European countries introduced precaution measures including limited socializing of people, closing of most public services and introducing mandatory facial protection. The hope for a return to the life before the pandemic is the development of an effective and safe vaccine against SARS-CoV-2 which would presumably reduce the incidence of severe forms of COVID-19 and prevent the massive spread of the disease. At the end of November, we have 13 clinical trials in phase III involving SARS-CoV-2 vaccines based on inactivated viruses, recombinant non-pathogenic viral vectors and proteins. The first mRNA-based vaccine is currently being evaluated in phase II/III clinical trial and is already being distrib-uted and applied to high-risk population in the United Kingdom, the United States, and Israel, followed by the countries of the European Union, including the Czech Republic. In the review article we present currently ongoing clinical studies with a special focus on the phase III clinical trials and discuss the mechanisms of action of each type of vaccine. © 2020, Czech Medical Association J.E. Purkyne. All rights reserved.