Rare Diseases Symptoms Automatic Extraction
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Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience.
[systemic capillary leak syndrome]
To
determine
clinical
features
,
natural
history
,
and
outcome
of
a
well-defined
cohort
of
25
consecutive
patients
with
idiopathic
systemic
capillary
leak
syndrome
(
SCLS
)
evaluated
at
a
tertiary
care
center
.
Records
of
patients
diagnosed
as
having
SCLS
from
November
1
,
1981
,
through
April
30
,
2008
,
were
reviewed
.
Descriptive
statistics
were
used
to
analyze
patient
demographics
,
clinical
features
,
complications
,
and
therapeutic
interventions
.
Of
the
34
patients
whose
records
were
reviewed
,
25
fulfilled
all
diagnostic
criteria
for
SCLS
.
The
median
age
at
diagnosis
of
SCLS
was
44
years
.
Median
follow-up
of
surviving
patients
was
4
.
9
years
,
and
median
time
to
diagnosis
from
symptom
onset
was
1
.
1
years
(
interquartile
range
,
0
.
5
-
4
.
1
years
)
.
Flulike
illness
or
myalgia
was
reported
by
14
patients
(
56
%
)
at
onset
of
an
acute
attack
of
SCLS
,
and
rhabdomyolysis
developed
in
9
patients
(
36
%
)
.
Patients
with
a
greater
decrease
in
albumin
level
had
a
higher
likelihood
of
developing
rhabdomyolysis
(
p
=
.
03
)
.
Monoclonal
gammopathy
,
predominantly
of
the
IgG-κ
type
,
was
found
in
19
patients
(
76
%
)
.
The
progression
rate
to
multiple
myeloma
was
0
.
7
%
per
person-
year
of
follow-up
.
The
overall
response
rate
to
the
different
therapies
was
76
%
,
and
24
%
of
patients
sustained
durable
(
>
2
years
)
complete
remission
.
The
estimated
5
-
year
overall
survival
rate
was
76
%
(
95
%
confidence
interval
,
59
%
-
97
%
)
.
Systemic
capillary
leak
syndrome
,
a
rare
disease
that
occurs
in
those
of
middle
age
,
is
usually
diagnosed
after
a
considerable
delay
from
onset
of
symptoms
.
The
degree
of
albumin
decrement
during
an
attack
correlates
with
development
of
rhabdomyolysis
.
A
reduction
in
the
frequency
and
/
or
the
severity
of
attacks
was
seen
in
nearly
three
-fourths
of
patients
who
were
offered
empirical
therapies
.
The
rate
of
progression
to
multiple
myeloma
appears
to
be
comparable
to
that
of
monoclonal
gammopathy
of
undetermined
significance
.
Diseases
Validation
Diseases presenting
"rhabdomyolysis"
symptom
adrenomyeloneuropathy
dystrophic epidermolysis bullosa
focal myositis
inclusion body myositis
pyomyositis
pyruvate dehydrogenase deficiency
scrub typhus
systemic capillary leak syndrome
This symptom has already been validated
