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Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment.
[systemic capillary leak syndrome]
Systemic
capillary
leak
syndrome
(
SCLS
)
is
a
rare
condition
characterized
by
unexplained
episodic
capillary
hyperpermeability
due
to
a
shift
of
fluid
and
protein
from
the
intravascular
to
the
interstitial
space
.
This
results
in
diffuse
swelling
,
weight
gain
,
and
renal
shut-down
.
From
the
first
publication
in
1960
,
only
34
cases
have
been
reported
.
To
collate
enough
patients
to
observe
the
natural
history
of
the
disease
and
evaluate
the
efficacy
of
empiric
treatments
.
Multicentric
retrospective
study
.
Thirteen
patients
(
6
women
and
7
men
)
were
collated
with
a
mean
follow-up
of
6
.
4
years
.
Eight
patients
are
still
alive
after
a
mean
of
5
.
6
years
(
range
1
to
15
)
.
Three
patients
out
of
the
11
who
were
not
lost
to
follow-up
died
;
1
during
an
attack
and
2
because
of
a
progression
towards
multiple
myeloma
.
Our
series
shows
an
improvement
in
the
prognosis
of
SCLS
due
most
likely
to
improved
management
during
attacks
.
Some
patients
'
disease
could
evolve
into
a
multiple
myeloma
.
Treatment
is
still
empiric
and
no
prophylactic
therapy
,
including
terbutaline
associated
with
aminophylline
,
has
clearly
proven
its
efficacy
.
