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Research Article
Anete Sevciovic Grumach
Faculdade de Medicina do ABC
Corresponding Author
ORCiD: https://orcid.org/0000-0002-9803-0309
License:
This work is licensed under a CC BY 4.0 License. Read Full License
Purpose
Hereditary angioedema (HAE) is a rare genetic disease with hyperactivated contact and kallikrein-kinin systems leading to bradykinin (BK) release and edema. SARS-CoV-2 infection results in inflammatory exacerbation. C1 inhibitor (C1-INH) deficiency could aggravate clinical outcomes, with HAE patients at a greater risk of adverse outcomes of COVID-19, however, data are still limited. Our aim was to characterize the course and severity of COVID-19 in patients with HAE.
Methods
Latin American HAE reference centers evaluated SARS-CoV-2 infection in this population. Patients with confirmed diagnosis of HAE with (HAE-C1-INH) or without C1-INH deficiency (HAE-nC1-INH) were included. HAE symptomatology and the course of COVID-19 were characterized with the application of a questionnaire.
Results
66 patients from 10 countries (HAE-C1-INH 80,3%; HAE-nC1-INH 19.6%) were reported with SARS-CoV-2 infection. Comorbidities were absent in 69.7% of the patients and obesity present in 12.1%. Attacks occurred in 45.5% of patients with HAE during SARS-CoV-2 infection. Long term prophylaxis was reported in 52% (34/66) of HAE patients. Complete cure was observed in 61 patients (92.4%), pulmonary sequelae in 4 and death in one HAE-C1-INH patient. The cause of death was septic shock secondary to bacterial pulmonary coinfection. Disease progression was not impacted by sex, therapy or type of HAE (p = 0.803).
Conclusion
Attacks occurred in almost half of HAE patients suggesting that SARS-CoV-2 infection is a trigger. HAE did not represent a risk factor for a worse outcome of COVID-19, even in use of androgens.
Keywords
Hereditary angioedema, C1 inhibitor, COVID-19, SARS-CoV-2, Androgen, Risk factor, Trigger, Hereditary angioedema with normal C1-INH
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This preprint is under consideration at Journal of Clinical Immunology. A preprint is a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Research Article
Anete Sevciovic Grumach
Faculdade de Medicina do ABC
Corresponding Author
ORCiD: https://orcid.org/0000-0002-9803-0309
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Under Review
Version 1
Posted 07 Apr, 2021
No community comments so far
Reviewers invited
Invitations sent on 30 Mar, 2021
First submitted
On 30 Mar, 2021
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Immunology
License:
This work is licensed under a CC BY 4.0 License. Read Full License
Purpose
Hereditary angioedema (HAE) is a rare genetic disease with hyperactivated contact and kallikrein-kinin systems leading to bradykinin (BK) release and edema. SARS-CoV-2 infection results in inflammatory exacerbation. C1 inhibitor (C1-INH) deficiency could aggravate clinical outcomes, with HAE patients at a greater risk of adverse outcomes of COVID-19, however, data are still limited. Our aim was to characterize the course and severity of COVID-19 in patients with HAE.
Methods
Latin American HAE reference centers evaluated SARS-CoV-2 infection in this population. Patients with confirmed diagnosis of HAE with (HAE-C1-INH) or without C1-INH deficiency (HAE-nC1-INH) were included. HAE symptomatology and the course of COVID-19 were characterized with the application of a questionnaire.
Results
66 patients from 10 countries (HAE-C1-INH 80,3%; HAE-nC1-INH 19.6%) were reported with SARS-CoV-2 infection. Comorbidities were absent in 69.7% of the patients and obesity present in 12.1%. Attacks occurred in 45.5% of patients with HAE during SARS-CoV-2 infection. Long term prophylaxis was reported in 52% (34/66) of HAE patients. Complete cure was observed in 61 patients (92.4%), pulmonary sequelae in 4 and death in one HAE-C1-INH patient. The cause of death was septic shock secondary to bacterial pulmonary coinfection. Disease progression was not impacted by sex, therapy or type of HAE (p = 0.803).
Conclusion
Attacks occurred in almost half of HAE patients suggesting that SARS-CoV-2 infection is a trigger. HAE did not represent a risk factor for a worse outcome of COVID-19, even in use of androgens.
This is a list of supplementary files associated with this preprint. Click to download.
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