Abstract
OBJECTIVE: Hereditary angioedema (HAE) is a rare disease that presents with recurrent attacks that can be triggered by stress or other emotions. The emotional impact of HAE is well-documented, although mainly via self-reported questionnaires. In this study, we used physiological measures to investigate stress regulation in patients with HAE and compared them to patients with immune thrombocytopenia (ITP), a disease that also progresses with attacks but without emotional triggers. METHODS: Twenty-six adult patients with HAE or ITP (13 each) were included in the study. Patients were asked to perform several computerized tasks that activated different branches of the autonomic nervous system (ANS). Heart rate variability and electrodermal activity were measured during the experimental tasks and a medical consultation that followed the experiments. RESULTS: Patients with HAE did not differ from patients with ITP in their physiological responses. In the reactivity tasks, a non-significant correlation significance was observed between reaction times (RTs) and peak spread in the HAE and ITP groups only for male patients and in a different way for HAE (negative correlation) and ITP (positive correlation). During medical consultations, an association between the physician’s perception of the patient’s stress and the patient’s physiological measures of stress was found only in female HAE patients, but without significance. CONCLUSIONS: The ANS of patients with HAE and ITP was similarly and abnormally activated, with a possible effect by sex. These results shed new light on the role of emotional regulation in chronic diseases. TRIAL REGISTRATION: This article does not report the results of a health care intervention on human participants. The research was not preregistered in any registry. The analysis plan was not preregistered in an independent, institutional registry. Data, analytic methods, and study materials are not made directly available. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13030-025-00348-6.