Researchers presented a case report in Cureus of a patient with hereditary angioedema who died shortly after contracting coronavirus disease 2019 (COVID-19).

Hereditary angioedema is characterized by recurrent swelling via bradykinin or dysregulation of the histaminergic response. This then causes the inflammatory cascade to proceed unchecked, giving rise to vasodilation and vascular permeability, the very conditions needed for edema attacks to occur. 

Coincidentally, the pathophysiology of SARS-Cov-2 works in a similar fashion; the actions of des-arginine-bradykinin are left unchecked because of depleted reserves of angiotensin-converting enzyme 2. The case study demonstrates how inflammatory pathways are activated in a patient with both these conditions. 


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The case report details a 51-year-old woman who presented to the hospital due to respiratory distress. She had a previous diagnosis of hereditary angioedema. A rapid antigen test revealed that she was positive for COVID-19. Her physicians were convinced that her acute hypoxic respiratory failure was likely secondary to COVID-19 pneumonia. The patient was intubated on an emergency basis. 

Read more about hereditary angioedema etiology 

Further medical history obtained from the patient’s family revealed that she received a monthly regime of immunoglobulins. In addition, she was also on C1 esterase inhibitor for angioedema prophylaxis due to her C1 esterase inhibitor deficiency. 

The plan was straightforward: treat COVID-19 pneumonia using dexamethasone and start the patient on broad-spectrum antibiotics, which were administered through a central line. She also received deep vein thrombosis prophylaxis with apixaban and seizure prophylaxis with levetiracetam. 

Her physicians decided it was best to initiate a short-term prophylaxis protocol in view of her hereditary angioedema. She continued to receive a standard regimen of subcutaneous C1 esterase inhibitor (Haegarda®); icatibant was kept at the bedside in case of an emergent angioedema attack. 

A few days later, the patient’s clinical condition worsened. She developed acute respiratory distress syndrome, worsening hypotension, and unsuccessful spontaneous breathing trials. She died shortly after. 

“The pathogenesis of [hereditary angioedema] overlaps with that of COVID-19, thereby elucidating potential mechanisms which may be affected to alter the natural course of the disease,” the authors of the study wrote. “Targeting inflammatory cascades and their constituents, such as bradykinin, may lead to a potent treatment for COVID-19 and prevent angioedema flares in the process.”

Reference

Park EG, Silvano Z, Gregory GE, Ghaly M, Case J. The interplay of COVID-19 and hereditary angioedema: preventing an acute attack. Cureus. 2022;14(9):e29189. doi:10.7759/cureus.29189