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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
"proximal weakness"
symptom
inclusion body myositis
neuralgic amyotrophy
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