29
3.41 Treatment of Acute Attacks
C1-INH concentrate is considered safe and effective and
C1-INH replacement therapy is recommended as a first-line
treatment for acute attacks in the management of HAE.
Other therapeutic options recommended for acute attacks
are recombinant C1-INH concentrates, the bradykinin
receptor antagonist icatibant, and the kallikrein inhibitor
ecallantide.
11,71
The consistent efficacy and safety of C1-INH concentrate
for the treatment of acute attacks, even during pregnancy,
lactation, and childhood, were also acknowledged in the
evidence-based recommendations for the therapeutic
management of HAE.
67
In pediatric patients, only C1-INH
concentrate is recommended.
69,71
3.42 Short- and Long-term Prophylaxis
It is recommended that for short-term prophylaxis (pre-
procedure prevention), C1-INH concentrate should be
immediately available whenever possible.
For major procedures or intubation, administration of 10
to 20 international units (IU)*/kg body weight or 1,000 IU
1 to 6 hours prior to the procedures is recommended. In
case of immediate availability of C1-INH concentrate, no
prophylaxis is needed before minor manipulations. Only
if C1-INH concentrate is not available, the International
Consensus panel recommends, with reservations, the use
of attenuated androgens for short-term prophylaxis.
11,71
For patients with more than 1 severe attack per month, and
if treatment for acute attacks is not sufficiently effective or
not available, the International Consensus panel suggests
to consider long-term prophylaxis with C1-INH concentrate,
antifibrinolytics, or attenuated androgens.
11
The WAO
guidelines, however, differ in that they consider the use of
androgens for long-term prophylaxis as critical and advise
against the use of antifibrinolytics for this indication.
71
The
international evidence-based consensus guidelines also
recommend C1-INH concentrate for long-termprophylaxis.
67
* The potency of C1-esterase inhibitor is expressed in International Units (IU), which are related
to the current WHO Standard for C1-esterase inhibitor products.
3.43 Self-administration
All guidelines agree on the benefit of offering home C1-INH
self-infusion programs to patients.
11,71
The International
Home Therapy Consensus Document recommends early
treatment of attacks at all sites with individualized doses of
C1-INH concentrate and advises considering every patient
with HAE for home therapy and self-administration training
to enable patients to manage their symptoms proactively,
and to reduce disruption in living a healthy and productive
life.
68
For participating in a self-infusion program, the availability
of a “home-therapy partner” is desirable but not a pre-
requisite, and pregnancy, lactation or extremes of age are
no contraindications, given the patients or a responsible
adult can safely perform administration.
68
In the case of laryngeal attacks, patients are strongly ad-
vised to seek emergency medical assistance after self-
administration.
68
3.44 Treatment During Pregnancy and Lactation
Pregnancy, like any other state of fluctuation of female
hormones, can influence the frequency and severity of
HAE attacks. Clinical symptoms, particularly abdominal
attacks, often worsen during the first
72
and third
73
trimesters
of pregnancy, which are associated with an increase of
estrogen levels. The increase in frequency and severity of
abdominal attacks during pregnancies may be partly due to
displacement of abdominal organs resulting from growth
of the uterus and fetal movements.
72,73
In line with theWAO guideline, the International Consensus
and Practical Guidelines on the Gynecologic and Obstetric
Management of Female Patients with HAE recommend
C1-INH concentrate as the preferred therapy during preg-
nancy for acute attacks.
70
It is also recommended as the
treatment of choice for acute attacks and short- and long-
term prophylaxis during lactation.
70,71
HAE
