Hereditary angioedema with C1 inhibitor deficiency: clinical presentation and quality of life of 193 French patients.
BACKGROUND
Hereditary angioedema (HAE) is a rare and potentially life-threatening disease. New specific treatments are available.


OBJECTIVE
To identify patients' features and patients' best therapeutic option.


METHODS
A 1-year, multicenter, retrospective study was performed. The primary objective was to examine the clinical presentation of HAE. Secondary objectives included patient characteristics, management of HAE over 12 months, and health-related quality of life using the SF-36v2 questionnaire.


RESULTS
One hundred ninety-three patients were included, and 69.4% were women. In the 12-month period, the mean number of HAE attacks was 7.6. Among the 568 reported attacks, localizations were the abdomen (57.1%), peripheral limbs (42.5%), upper airway (7.9%), and face (6.9%); 31.6% of attacks were severe and occurred statistically more often in women (P < .02). Compared with a population of allergic patients, all age- and sex-adjusted scores were significantly lower in patients with HAE (P < .05) except for the physical component summary. Health-related quality of life negatively correlated with the annual number of attacks and was markedly altered for patients having more than 5 attacks per year (P < .05 for all dimensions).


CONCLUSIONS
HAE is a severe disease that places a heavy burden on quality of life.
Department of Internal Medicine, University Hospital, Grenoble, France. Electronic address: LBouillet@chu-grenoble.fr.
Laurence
L
Bouillet
David
D
Launay
Olivier
O
Fain
Isabelle
I
Boccon-Gibod
Jérôme
J
Laurent
Ludovic
L
Martin
Vincent
V
Montauban
K
K
Finck
Stéphane
S
Bouée
Anne
A
Gompel
Gisèle
G
Kanny
D000328:Adult; D050718:Complement C1 Inhibitor Protein; D005260:Female; D005602:France / Q000453:epidemiology; D056829:Hereditary Angioedema Types I and II / Q000453:epidemiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D012189:Retrospective Studies; D055815:Young Adult
D050718:Complement C1 Inhibitor Protein