Rare Diseases Symptoms Automatic Extraction
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Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression.
[systemic capillary leak syndrome]
A
female
patient
in
her
60
s
presented
with
a
history
of
malaise
,
chills
,
headache
and
vomiting
.
She
was
in
shock
on
presentation
with
a
high
haematocrit
and
a
low
albumin
with
evidence
of
rhabdomyolysis
.
Severe
limb
and
truncal
oedema
developed
with
worsening
hypotension
leading
to
intensive
care
unit
admission
for
multiple
organ
support
.
Extensive
radiological
,
microbiological
and
immunological
work
up
was
negative
with
the
exception
of
a
monoclonal
gammopathy
.
A
review
of
patient
investigations
led
to
a
diagnosis
of
Clarkson
's
disease
.
Treatment
with
high
-dose
methylprednisolone
and
intravenous
immunoglobulins
led
to
a
rapid
decline
in
the
creatine
kinase
(
CK
)
level
and
vasopressor
requirements
.
The
patient
was
discharged
home
on
long
-term
terbutaline
and
has
made
a
good
recovery
.
