Researchers presented 3 cases that demonstrated successful approaches to long-term prophylactic therapy in patients with hereditary angioedema. The case series was published in Frontiers in Allergy.

Hereditary angioedema is characterized by frequent attacks of laryngeal edema, skin swellings, and abdominal pain. Patients with the disease may experience severe disability; hereditary angioedema attacks can also be life-threatening. 

One of the chief goals of treatment is to allow patients to lead lives that are as normal as possible. This often translates into long-term prophylaxis to prevent the occurrence of edema attacks. A number of intravenous plasma-derived human C1 inhibitor concentrates, such as lanadelumab and berotralstat, have been demonstrated to be efficacious in this regard. 

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The authors of this study analyzed 3 case studies of patients with hereditary angioedema who received long-term prophylaxis. One of these cases details a 13-year-old girl who was diagnosed with hereditary angioedema at the age of 2 years. Suspicion of the diagnosis was raised because of a positive family history. 

Read more about hereditary angioedema treatment 

The child’s first attack involved facial swelling, which took place when she was 2.5 years of age. From then on, she began to experience recurrent attacks, affecting various parts of her body, including the face, gastrointestinal tract, and extremities. Therapy was often delayed because these attacks frequently happened during the night and during hospitalization, and unnecessary diagnostic procedures were carried out despite her known diagnosis of hereditary angioedema. 

Subcutaneous icatibant was prescribed for her attacks, but that did not prevent her from experiencing further attacks. She typically experienced 3 to 4 attacks per month. 

Her physicians then decided to pursue a strategy of long-term prophylaxis with a subcutaneous C1 inhibitor, with the initial dose being 24 U/kg twice weekly. The patient was compliant with the therapy and experienced fewer attacks per month. 

“In comparison, this patient experienced 2.67 attacks per month within the 3 months prior to long-term prophylaxis compared to 0.13 attacks per month during the steady state with this well-tolerated prophylactic treatment,” the authors of the study wrote. “The reduction of attack rate with the low dose was 95%.”

Her disease was well controlled using this long-term prophylactic treatment regimen. 

This case study, as well as the others presented in this series, highlight the efficacy of long-term prophylaxis as a therapeutic strategy to reduce the frequency of attacks in patients with hereditary angioedema. 


Andarawewa S, Aygören-Pürsün E. Individual approach to long-term therapy in patients with hereditary angioedema (HAE-C1-INH): a case seriesFront Allergy. 2022;3:949387. doi:10.3389/falgy.2022.949387