D2.318 - Triggers and prodromal indicators of angioedema attacks in hereditary angioedema patients

Hereditary angioedema type 1 and 2 (HAE-1/2) is a rare autosomal dominant disorder caused by mutations in the C1 inhibitor gene (SERPING1), leading to recurrent, unpredictable episodes of subcutaneous and submucosal swelling. These episodes can affect the extremities, gastrointestinal tract, airway, and other tissues. Triggers and prodromes for HAE-1/2 attacks vary widely among patients, and identifying these factors is crucial for improving management of the disease. We analyzed the triggers and prodromes in 59 HAE-1/2 patients, emphasizing their prevalence and patterns.

Data from 59 HAE-1/2 patients were analyzed, focusing on the triggers and prodromes associated with their attacks. Triggers evaluated included trauma, stress, viral infections, menstruation, oestrogens, and others. Prodromes analyzed included erythema, abdominal discomfort, asthenia, irritability, and others. Additionally, the prevalence of single versus multiple triggers and prodromes was assessed, along with patients reporting no identifiable patterns.

   ∙ Triggers:

∙Trauma was the most frequently reported trigger (31.7%), followed by stress (21.7%) and viral infections (16.7%).

∙Menstruation (14.2%), oestrogens (4.2%), and "no identifiable triggers" (5.8%) were less commonly reported.

∙35 patients (60.34%) reported multiple triggers, while 5 (8.62%) had no identifiable triggers.

∙Two patients had more than three trigger options, while 17 reported more than two and 35 reported more than one trigger option.

    ∙ Prodromes:

∙The most common prodrome was "no identifiable prodromes" (29.3%), followed by abdominal discomfort (25.3%) and erythema (24.0%).

∙Asthenia (13.3%), irritability (1.3%), and other prodromes (6.7%) were also observed.

∙26 patients (46.43%) reported single prodromes, while 11 (19.64%) experienced multiple prodromes.

∙11 patients had more than one prodrome option, while only one patient had more than two options.

The correlation analysis between patients and risk factors (r = 0.079, p = 0.586) indicates that multiple triggers are not a significant risk factor for increased attack frequency. Specific trigger subcategories and combinations were not evaluated, and logistic regression was not performed.

Trauma (31.7%) and stress (21.7%) are the primary triggers for HAE-1/2 attacks in our cohort, while abdominal discomfort (25.3%) and erythema (24.0%) were the leading prodromes. Notably, 35 patients reported  more than one trigger option, 11 had more than one prodrome option, and some patients identified no clear triggers (8.62%) or prodromes (33.93%).