Rare Diseases Symptoms Automatic Extraction
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[Inclusion body myositis: series of 30 cases from a Brazilian tertiary center].
[inclusion body myositis]
To
describe
a
series
of
30
consecutive
patients
with
inclusion
body
myositis
(
IBM
)
from
our
tertiary
center
,
from
1982
to
2012
.
All
patients
fulfilled
the
criteria
of
Griggs
et
al
.
(
1995
)
for
IBM
.
T
he
mean
age
of
patients
at
disease
onset
was
60
.
8
�
11
.
9
years
with
disease
duration
of
8
.
0
�
5
.
2
years
.
Eighty
%
of
patients
were
Caucasian
,
with
similar
distribution
between
genders
.
Weight
loss
in
early
disease
was
present
in
less
than
a
quarter
of
cases
.
The
main
symptom
was
proximal
weakness
of
the
lower
limbs
followed
by
weakness
of
the
upper
(
proximal
and
/
or
distal
)
limbs
.
One
third
of
patients
had
dysphagia
,
whereas
dysphonia
was
present
in
16
.
7
%
,
arthralgias
in
6
.
7
%
,
moderate
dyspnea
symptoms
in
3
.
3
%
of
cases
.
All
patients
received
prednisone
(
1
mg
/
kg
/
day
)
.
Several
immunosuppressives
were
used
as
corticosteroid-sparing
according
to
tolerance
,
side
effects
and
/
or
refractoriness
.
Half
of
the
patients
still
in
follow-up
remained
stable
according
to
clinical
and
laboratory
data
during
the
study
.
There
were
four
cases
of
cancer
,
four
cases
associated
with
viral
infections
(
HIV
and
hepatitis
C
virus
)
and
three
deaths
(
two
because
of
sepsis
secondary
to
community
bronchopneumonia
,
and
one
because
of
congestive
heart
failure
)
.
This
is
the
first
Brazilian
series
of
cases
involving
large
sample
of
IBM
.
The
profile
of
the
patients
analyzed
in
this
study
was
comparable
to
those
profiles
described
in
literature
,
except
that
of
IBM
cases
of
our
population
are
equally
distributed
in
both
genders
and
the
interval
between
symptoms
onset
and
diagnosis
of
the
disease
was
relatively
short
.
It
is
relevant
to
note
the
high
frequency
of
neoplastic
diseases
and
chronic
viral
infections
in
our
population
,
reinforcing
the
need
for
specific
epidemiological
studies
to
verify
these
associations
,
once
it
is
poorly
described
in
the
literature
.
Diseases
Validation
Diseases presenting
"dyspnea"
symptom
acute rheumatic fever
allergic bronchopulmonary aspergillosis
carcinoma of the gallbladder
congenital diaphragmatic hernia
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
erdheim-chester disease
esophageal carcinoma
fabry disease
familial mediterranean fever
inclusion body myositis
liposarcoma
lymphangioleiomyomatosis
neuralgic amyotrophy
pleomorphic liposarcoma
primary effusion lymphoma
scrub typhus
systemic capillary leak syndrome
thoracic outlet syndrome
well-differentiated liposarcoma
This symptom has already been validated