Abstract
Background: Chronic histaminergic angioedema (CHA) is defined
as recurrent episodes of isolated angioedema (without hives) of unknown
cause that respond to the same treatment as chronic spontaneous
urticaria (CSU). Quality of life (QoL) studies have not been performed
for CHA, except those carried out in the context of CSU associated with
angioedema attacks (CSU-AE). Moreover, biomarkers for monitoring disease
activity in CHA have not been identified. We aim to describe the burden
of CHA and impact on patient QoL, compare the findings to those in
CSU-AE patients, and investigate biomarker associations with disease
severity and QoL parameters.
Methods: We performed a prospective multicenter study that
included 68 patients with CHA and 63 patients with CSU-AE. Demographic
and clinical variables were collected. Validated patient-reported
questionnaires were employed to analyze the quality of life and disease
activity. Blood and serological parameters, including blood cell count,
C-reactive protein, D-dimer and total IgE, were also analyzed.
Results: Angioedema disease activity was significantly higher
in CSU-AE patients (median AAS7, IQR: 1, [0–1]) than CHA patients
(0, [0–1]; p= 0.022). A considerable impact on QoL was found in
both groups, although significantly worse values were found for CSU-AE
(median AEQoL, IQR: 37, [10–65]; p=0.005). CHA patients were older
than CSU-AE patients, and female predominance was not observed.
Conclusions: Angioedema severity and QoL impacts are
significantly worse in CSU than in chronic histaminergic angioedema.
Angioedema should be included in severity urticaria scores (UAS) as well
as in specific quality of life urticaria scales.
Keywords: Angioedema, Chronic Histaminergic Angioedema, Chronic
Spontaneous Urticaria, Disease activity, Quality of Life