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[A case report of systemic capillary leak syndrome. Effective treatment with immunoadsorption and leukotriene antagonist].
[systemic capillary leak syndrome]
Systemic
capillary
leak
syndrome
(
SCLS
)
is
an
unusual
condition
characterized
by
periodic
leakage
of
plasma
proteins
through
the
capillary
wall
,
leading
to
hypoalbuminaemia
,
hypovolaemia
,
haemoconcentration
and
shock
.
The
pathogenesis
is
unknown
and
the
mortality
high
.
Various
prophylactic
treatments
have
been
tried
but
are
difficult
to
evaluate
because
of
the
unpredictable
course
of
the
disease
.
We
describe
one
patient
with
frequent
attacks
of
SCLS
,
that
did
not
respond
to
any
kind
of
treatment
regimens
over
a
period
of
nine
years
.
Subsequently
,
therapy
with
regular
immunoadsorption
of
plasma
proteins
and
a
leukotriene
receptor
antagonist
was
attempted
.
This
treatment
has
now
been
given
for
six
years
and
only
one
episode
of
capillary
leakage
has
occurred
.
The
effect
may
be
due
to
removal
of
pathogenic
immunoglobulins
or
immune
complexes
and
/
or
inhibition
of
leukotriene-induced
capillary
leakage
.
