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High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome.
[systemic capillary leak syndrome]
The
systemic
capillary
leak
syndrome
(
SCLS
)
,
also
known
as
Clarkson
's
disease
,
is
a
life-threatening
disorder
of
unknown
cause
.
It
is
characterised
by
recurrent
acute
episodes
of
hypotension
,
weight
gain
and
generalised
oedema
with
haemoconcentration
and
hypoproteinaemia
caused
by
paroxysmal
capillary
hyperpermeability
with
a
shift
of
plasma
fluid
from
the
intravascular
to
the
interstitial
space
.
We
report
the
case
of
a
40
-
year
-old
woman
with
chronic
SCLS
treated
with
high
-dose
intravenous
immunoglobulins
,
after
a
prophylactic
therapy
with
theophylline
and
terbutaline
was
poorly
tolerated
and
failed
to
decrease
the
frequency
and
severity
of
the
attacks
sufficiently
.
