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[Systemic capillary leak syndrome].
[systemic capillary leak syndrome]
A
man
aged
61
had
recurrent
attacks
of
severe
shock
.
The
episodes
were
preceded
by
symptoms
such
as
a
runny
nose
,
epigastric
discomfort
with
nausea
,
vertigo
,
orthostatism
and
sometimes
light
fever
.
During
the
attacks
there
were
marked
hypotension
,
a
strong
rise
of
the
haematocrit
,
a
decrease
of
the
protein
and
albumin
concentrations
in
the
blood
and
prerenal
kidney
failure
.
In
addition
,
there
was
a
paraprotein
,
type
IgG-kappa
.
The
shock
every
time
responded
rapidly
to
intravenous
administration
of
fluid
and
was
followed
by
a
period
of
substantial
polyuria
.
The
pattern
was
characteristic
of
systemic
capillary
leak
syndrome
,
a
rare
but
frequently
fatal
disease
characterized
by
episodes
of
unexplained
extravasation
of
plasma
.
The
aetiology
and
pathogenesis
are
unknown
.
Attacks
are
suppressed
by
supportive
therapy
(
administration
of
fluids
,
inotropics
)
and
future
attacks
may
be
prevented
by
the
intake
of
terbutaline
and
theophylline
.
The
systemic
capillary
leak
syndrome
should
be
considered
in
the
differential
diagnosis
of
idiopathic
and
anaphylactic
shock
.
