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Chronic systemic capillary leak syndrome. Report of a case and review of the literature.
[systemic capillary leak syndrome]
Systemic
capillary
leak
syndrome
(
SCLS
)
is
a
rare
condition
characterized
by
recurrent
episodes
of
generalized
oedema
and
severe
hypotension
,
associated
with
paraproteinaemia
.
In
addition
to
the
acute
form
,
a
few
cases
of
chronic
SCLS
have
been
reported
.
We
describe
a
64
-
year
-old
woman
who
was
hospitalized
because
of
a
6
-
month
history
of
progressive
generalized
oedema
with
pericardial
and
pleural
effusions
,
associated
with
a
serum
paraprotein
.
Clinical
and
laboratory
findings
were
consistent
with
a
chronic
form
of
SCLS
.
Treatment
with
prednisone
,
furosemide
and
theophylline
was
started
,
which
led
to
a
gradual
improvement
in
2
weeks
and
a
persistent
remission
after
9
months
.
This
report
indicates
that
SCLS
may
occur
in
a
chronic
form
,
which
seems
to
be
responsive
to
a
therapeutic
regimen
with
prednisone
,
furosemide
,
and
theophylline
.
Diseases
Validation
Diseases presenting
"recurrent episodes"
symptom
acute rheumatic fever
adrenomyeloneuropathy
benign recurrent intrahepatic cholestasis
child syndrome
cholangiocarcinoma
cystinuria
familial mediterranean fever
hydrocephalus with stenosis of the aqueduct of sylvius
kabuki syndrome
neuralgic amyotrophy
pyruvate dehydrogenase deficiency
systemic capillary leak syndrome
triple a syndrome
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