Disease expression in women with hereditary angioedema.
OBJECTIVE
Fluctuations in sex hormones can trigger angioedema attacks in women with hereditary angioedema. Combined oral contraceptive therapies, as well as pregnancy, can induce severe attacks. The course of angioedema may be very variable in different women.


METHODS
Within the PREHAEAT project launched by the European Union, data on 150 postpubertal women with hereditary angioedema were collected in 8 countries, using a patient-based questionnaire.


RESULTS
Puberty worsened the disease for 62%. Combined oral contraceptives worsened the disease for 79%, whereas progestogen-only pills improved it for 64%. During pregnancies, 38% of women had more attacks, but 30% had fewer attacks. Vaginal delivery was usually uncomplicated. Attacks occurred within 48 hours in only 6% of cases. Those more severely affected during menses had more symptoms during pregnancies, suggesting a hormone-sensitive phenotype for some patients.


CONCLUSIONS
The course of angioedema in women with C1 inhibitor deficiency is affected by physiologic hormonal changes; consequently, physicians should take these into account when advising on management.
Department of Internal Medicine, Grenoble University Hospital, Grenoble, France.
Laurence
L
Bouillet
Hilary
H
Longhurst
Isabelle
I
Boccon-Gibod
Konrad
K
Bork
Christophe
C
Bucher
Anette
A
Bygum
Teresa
T
Caballero
Christian
C
Drouet
Henriette
H
Farkas
Christian
C
Massot
Erik W
EW
Nielsen
Denise
D
Ponard
Marco
M
Cicardi
D000328:Adult; D054179:Angioedemas, Hereditary / Q000503:physiopathology*; D003277:Contraceptives, Oral, Combined / Q000009:adverse effects; D036861:Delivery, Obstetric / Q000009:adverse effects; D005260:Female; D006728:Hormones / Q000502:physiology; D006801:Humans; D008598:Menstruation / Q000502:physiology; D008875:Middle Aged; D011247:Pregnancy; D011248:Pregnancy Complications / Q000503:physiopathology; D011374:Progesterone / Q000494:pharmacology; D011627:Puberty / Q000502:physiology
D003277:Contraceptives, Oral, Combined; D006728:Hormones; D011374:Progesterone