23
The following factors have been identified as
potentially eliciting attacks:
•
Hormonal fluctuations and estrogen
5,8,10,11,22,30,34,41,42
•
Oral-pharyngeal surgeries, dental procedures, and
intubation
5,8,10,11,38
•
Infections
5,8,11,30,34,42-44
•
Mechanical stress, physical exertion, or minor
trauma
5,8,10,11,30,34,41,42
•
Emotional stress or excitement
5,8,10,11,30,34,41,42
•
Medications, such as estrogen-containing contra-
ceptives and angiotensin-converting enzyme (ACE)
inhibitors
5,8,10,11,22,41,42
•
Exposure to cold
5
•
Specific foods or drinks, such as eggs, beer, wine,
pineapple
5,41
•
Insect stings
41
Hormonal Fluctuations and Estrogen
Menstruation has long been recognized as a trigger for
episodes of angioedema in some women.
5,10,34
Bouillet
et al reported on the effects of hormonal triggers, oral
contraceptives, and pregnancy on HAE symptoms in
150 postpubertal women from 8 countries.
22
Puberty
exacerbated the disease for 62% of the women. Likewise,
estrogen-containing contraceptives worsened HAE
symptoms for 79% of women, but progestogen improved
symptoms for 64% of women. Pregnancy aggravated the
frequency of attacks for some women and improved it for
others. However, only 13% of women had improvement
in their disease symptoms at menopause.
22
Oro-pharyngeal Surgeries, Dental Procedures,
and Intubation
Tooth extractions, tonsillectomies, and intubation during
general anesthesia have been known to trigger some-
times fatal laryngeal attacks.
5,38
Bork et al evaluated
the occurrence of facial swellings and laryngeal edema
following a total of 705 tooth extractions in 171 patients.
Facial swelling, or potentially life-threatening laryngeal
edema, or both occurred in 124 of 577 tooth extractions
(21.5%) without prophylaxis.
45
Infections
Various infections can cause attacks. For example, mono-
nucleosis and upper respiratory infections have been shown
to trigger laryngeal attacks in some individuals with HAE.
34,46
Farkas et al reported that 36.8% of 49 children with HAE
had airway infections as precipitating events.
34
Likewise,
Helicobacter pylori
infections can prompt abdominal
attacks.
43,44
Eradication of
Helicobacter pylori
in patients
with HAE significantly reduced the number of abdominal
attacks they experienced.
43,44
Mechanical Stress, Physical Exertion,
or Minor Trauma
Minor trauma can also precipitate HAE attacks. Farkas et al
found that mechanical trauma was the most common
provoking factor for angioedema episodes in children.
32
Frank also reported that repetitive tasks, such as sustained
gardening, lawn mowing, or using sewing shears can elicit
attacks in some individuals.
5,10
Even prolonged sitting or
standing has been implicated.
5
3.16 Possible Autoimmune Effects
of C1-INH Deficiency
Although most individuals with HAE are otherwise relatively
healthy, it does appear that people with C1-INH deficiency
may have a higher rate of autoimmune diseases, such as
systemic lupus, glomerulonephritis, Sjögren’s syndrome,
rheumatoid arthritis, and other medical conditions.
30,47-54
In a systematic evaluation of 157 HAE patients, 19 patients
(12%) had an autoimmune disease,
49
while the prevalence of
autoimmune diseases in the general population is estimated
to be slightly more than 3%.
55
It has been proposed that
C4 deficiency as a consequence of the C1-INH deficiency
may be a factor in the underlying pathophysiology of these
autoimmune conditions.
5
HAE
