A pilot study on the role of stress and emotional process in the variability of hereditary angioedema (HAE) in childhood and adolescence.

Background: Hereditary (HAE) is characterised by remarkable clini- cal variability and unpredictability of attacks. Previous studies suggested that stress and emotions can trigger HAE attacks. We carried out a study on 11 patients aged 4–17 years as well as their parents, to explore the connection between psychological factors and clinical symp- toms of HAE. Methods: We examined the psychopatho- logical risk of the patients, the connection between stress and illness and how the par- ents interpret the causes of the attacks. The Child Behaviour Check-List (CBCL) questionnaire was administered to 11 par- ents to evaluate the psychopathological risk. The young patients completed the Coddington Life Events Scale (CLES) to assess the level of perceived stressful events. Finally, semi-structured interviews were administered to the parents to explore their interpretations of the factors responsi- ble for the onset of the attacks. Results: On the CBCL scales more than one half of the patients achieved critical scores: five patients in the syndrome scale (3 of them in thought disorders); six patients in the scales dsm-oriented (anxiety and somatic complaints); six of them in the activity scale (competence scale). In the CLES 10 patients out of 11 were above the normal levels of stress, but frequency of attacks was not proportionally increased. The interviews show that 10 families out of 11 consider emotions to play a part in the expression of HAE, even if interpretation of them as symptoms, triggers or conse- quences of HAE differs between families. Conclusion: This pilot study confirms a connection between emotional and stressful factors and the onset of HAE attacks, evehough a larger study is needed. The per- ception of stress in patients with HAE is above the norm and the recognition of the role of emotions is constant. These dimen- sions do not correlate sistematically with the variablility of the symptoms. It is likely that processes of emotion regulation play a role in the onset of the attacks.