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Treatment of the systemic capillary leak syndrome with terbutaline and theophylline. A case series.
[systemic capillary leak syndrome]
The
systemic
capillary
leak
syndrome
is
a
rare
idiopathic
disorder
characterized
by
recurrent
episodes
of
hypotension
and
hemoconcentration
due
to
sudden
transient
extravasation
of
10
%
to
70
%
of
plasma
.
Mortality
rates
5
years
after
diagnosis
have
been
reported
to
be
76
%
.
To
assess
the
efficacy
of
a
prophylactic
regimen
for
the
systemic
capillary
leak
syndrome
.
Case
series
.
Tertiary
referral
center
.
Eight
patients
followed
over
the
past
18
years
.
Oral
terbutaline
plus
aminophylline
or
theophylline
.
Long
-term
clinical
follow-up
.
During
a
median
follow-up
of
9
years
(
range
,
2
to
18
years
)
,
two
patients
(
25
%
)
died
:
one
during
an
acute
episode
and
one
of
complications
related
to
long
-term
corticosteroid
therapy
.
The
other
six
patients
are
alive
and
healthy
.
The
frequency
and
severity
of
the
episodes
decreased
by
a
median
of
30
-
fold
.
Recurrences
were
associated
with
decreased
serum
theophylline
levels
,
possibly
caused
by
enzyme
induction
or
autoinduction
.
The
extended-release
form
of
medication
was
more
successful
.
Sympathomimetic
side
effects
were
significant
.
A
regimen
of
terbutaline
and
theophylline
seems
to
be
effective
prophylaxis
against
the
systemic
capillary
leak
syndrome
.
Maintenance
of
therapeutic
drug
levels
was
associated
with
favorable
results
.
