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Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome).
[systemic capillary leak syndrome]
The
systemic
capillary
leak
syndrome
(
SCLS
)
is
a
rare
disorder
characterized
by
transient
episodes
of
hypotensive
shock
and
anasarca
thought
to
arise
from
reversible
microvascular
barrier
dysfunction
.
Although
the
high
prevalence
of
a
monoclonal
gammopathy
of
unknown
significance
in
SCLS
suggests
a
pathogenic
contribution
of
endogenous
immunoglobulins
,
the
mechanisms
of
vascular
hyperpermeability
remain
obscure
.
Herein
,
we
report
clinical
and
molecular
findings
on
23
patients
,
the
largest
SCLS
case
series
to
date
.
Application
of
episodic
SCLS
sera
,
but
neither
the
purified
immunoglobulin
fraction
nor
sera
obtained
from
patients
during
remission
,
to
human
microvascular
endothelial
cells
caused
vascular
endothelial
cadherin
internalization
,
disruption
of
interendothelial
junctions
,
actin
stress
fiber
formation
,
and
increased
permeability
in
complementary
functional
assays
without
inducing
endothelial
apoptosis
.
Intravenous
immunoglobulin
,
one
promising
therapy
for
SCLS
,
mitigated
the
permeability
effects
of
episodic
sera
.
Consistent
with
the
presence
of
endogenous
,
nonimmunoglobulin
,
circulating
permeability
factor
(
s
)
constrained
to
SCLS
episodes
,
we
found
that
vascular
endothelial
growth
factor
(
VEGF
)
and
angiopoietin
2
(
Ang
2
)
,
were
elevated
in
episodic
SCLS
sera
but
not
in
remission
sera
.
Ab
-based
inhibition
of
Ang
2
counteracted
permeability
induced
by
episodic
SCLS
sera
.
Comparable
experiments
with
anti-
VEGF
Ab
(
bevacizumab
)
yielded
less
interpretable
results
,
probably
because
of
endothelial
toxicity
of
VEGF
withdrawal
.
Our
results
support
a
model
of
SCLS
pathogenesis
in
which
nonimmunoglobulin
humoral
factors
such
as
VEGF
and
Ang
2
contribute
to
transient
endothelial
contraction
,
suggesting
a
molecular
mechanism
for
this
highly
lethal
disorder
.
Diseases
Validation
Diseases presenting
"vascular endothelial cadherin internalization"
symptom
systemic capillary leak syndrome
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