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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
.
Diseases
Validation
Diseases presenting
"female patient"
symptom
achondroplasia
adrenal incidentaloma
alexander disease
aniridia
aromatase deficiency
benign recurrent intrahepatic cholestasis
carcinoma of the gallbladder
coats disease
cohen syndrome
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentinogenesis imperfecta
epidermolysis bullosa simplex
esophageal squamous cell carcinoma
fabry disease
holt-oram syndrome
hydrocephalus with stenosis of the aqueduct of sylvius
kallmann syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
pyruvate dehydrogenase deficiency
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
x-linked adrenoleukodystrophy
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