Etiology and predictors of cluster-attacks following the treatment of acute hereditary angioedema (HAE) attacks

Hereditary angioedema is a disease, which leads to recurrent swelling of the skin and mucous membranes including the upper airway tract. Besides being deadly, these attacks can be debilitating, leading to a poor quality of life for patients. Clinicians are occasionally confronted with patients who have recurrent attacks despite treatment with C1-Inhibtor concentration or a B2-Receptor Inhibitor. The goal this study is to investigate repeat attacks which re-occur within a week (“Cluster-Attacks”) after treatment, to determine why they occur and with what factors they may be associated with, and hereby prevent their occurrence.

Methods:

We conducted a single-center mixed retrospective/prospective study data acquisition of all documented attacks from 2015 to 2017 from our HAE patient collective (n = 106).

Results:

85% of our patient collective agreed to participate in the study. 10% (n = 11) of these patients had Cluster-Attacks, with a total of 102 Cluster-Attacks. Approximately 66% of all Cluster-Attacks were caused by exogenous stimuli (35% due to psychological stress, 27% due to physical stimuli, and 4% due to Menstruation). 7% of patients who were receiving a prophylactic therapy had Cluster-Attacks. In comparison, 14% of patients who received an on-demand therapy had repeat attacks. A significant difference was found between the medication dosage and the length of time to the repeat attack between prophylactic group and the on-demand therapy (p = 0,014). The time-interval in the prophylactic group was found to be significantly longer.

Conclusions:

Cluster-Attacks are most commonly due to psychic stress and physical stimuli. In patients who are prone to cluster-attacks a prophylaxis should be considered.

Publication History

Publication Date:
23 April 2019 (online)