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Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature.
[systemic capillary leak syndrome]
The
systemic
capillary
leak
syndrome
(
SCLS
)
is
a
rare
condition
characterized
by
unexplained
episodic
attacks
of
systemic
capillary
hyperpermeability
accompanied
by
hypoalbuminemia
,
hemoconcentration
and
edema
.
Treatment
of
the
acute
phase
is
supportive
,
focusing
on
adequate
fluid
resuscitation
.
Many
agents
have
been
used
to
prevent
acute
attacks
,
including
corticosteroids
,
β
2
-
agonists
(
aminophylline
,
theophylline
,
or
terbutaline
)
,
infliximab
,
thalidomide
and
intravenous
immunoglobulin
(
IVIg
)
.
β
2
-
agonists
were
the
first
-line
maintenance
therapy
until
a
few
years
ago
.
In
more
recent
years
,
IVIg
became
common
first
-line
prophylactic
therapy
in
most
patients
with
benefits
at
the
dose
of
2
g
/
kg
once
a
month
.
We
report
the
case
of
a
49
-
year
-old
man
with
SCLS
treated
successfully
with
a
lower
dose
of
IVIg
(
1
g
/
kg
monthly
)
in
the
maintenance
phase
.
He
presented
no
acute
episodes
in
a
follow-up
of
28
months
.
We
describe
prophylactic
treatments
for
SCLS
in
literature
and
compare
our
patient
to
another
18
who
received
IVIg
in
follow-up
.
Diseases
Validation
Diseases presenting
"systemic capillary hyperpermeability"
symptom
systemic capillary leak syndrome
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