Researchers presented in Cureus the case report of a patient with hereditary angioedema (HAE) who had acute episodes possibly triggered by cocaine use. 

A 47-year-old man who was diagnosed with HAE 16 years previously presented for facial swelling, which had begun the previous evening. He denied taking any medications, including an epinephrine autoinjector, which he failed to locate. He also denied insect bites or eating new foods. 

The patient reported experiencing similar episodes before that required intubation, with the most recent episode happening just 4 months ago. He was administered dexamethasone, diphenhydramine, and famotidine. In addition, he was given a single dose of tranexamic acid, “a controversial treatment option for acute attacks of HAE,” according to the authors of the report. 

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Laboratory tests revealed that he had a low C4 and a C1 esterase inhibitor activity of 100%. In addition, his urine drug screen was positive for cocaine. A neck computed tomography (CT) revealed superficial subcutaneous edema without airway compromise. 

Read more about HAE etiology 

The patient was admitted into intensive care for close monitoring. His condition improved and he was discharged the next day. He was prescribed 200 mg of danazol twice daily for prophylaxis. He was scheduled for a follow-up 2 weeks later, but instead presented to the emergency department with shortness of breath and stridor on that day. He was intubated and extubated the next day. This time, his urine drug screen was positive for both cocaine and cannabinoids. He tested negative for Helicobacter pylori, a possible trigger for angioedema. 

The patient’s physicians requested that he stopped using illicit drugs, fearing that they were a trigger for his angioedema. He was discharged again with danazol. Social services sought to help him attain a year’s supply of free intravenous C1 esterase inhibitor, but he did not attend the necessary appointments and therefore did not receive the assistance he was entitled to.

Eight months later, he presented with the same issues—shortness of breath and stridor. This time, he was not intubated. There was no documentation of the use of danazol, which was previously prescribed. 

“In addition to discussing HAE medication accessibility, we share the first reported case of HAE episodes triggered by cocaine use,” the authors concluded. 


Welch NL, Peterson JA, Adams K. The price of a life: unveiling the struggle of living with hereditary angioedema. Cureus. Published online July 30, 2023. doi:10.7759/cureus.42699