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Comparative utility of LC3, p62 and TDP-43 immunohistochemistry in differentiation of inclusion body myositis from polymyositis and related inflammatory myopathies.
[inclusion body myositis]
Inclusion
body
myositis
(
IBM
)
is
a
slowly
progressive
inflammatory
myopathy
of
the
elderly
that
does
not
show
significant
clinical
improvement
in
response
to
steroid
therapy
.
Distinguishing
IBM
from
polymyositis
(
PM
)
is
clinically
important
since
PM
is
steroid-responsive
;
however
,
the
two
conditions
can
show
substantial
histologic
overlap
.
We
performed
quantitative
immunohistochemistry
for
(
1
)
autophagic
markers
LC
3
and
p
62
and
(
2
)
protein
aggregation
marker
TDP-
43
in
53
subjects
with
pathologically
diagnosed
PM
,
IBM
,
and
two
intermediate
T
cell-mediated
inflammatory
myopathies
(
polymyositis
with
COX
-negative
fibers
and
possible
IBM
)
.
The
percentage
of
stained
fibers
was
significantly
higher
in
IBM
than
PM
for
all
three
immunostains
,
but
the
markers
varied
in
sensitivity
and
specificity
.
In
particular
,
both
LC
3
and
p
62
were
sensitive
markers
of
IBM
,
but
the
tradeoff
between
sensitivity
and
specificity
was
smaller
(
and
diagnostic
utility
thus
greater
)
for
LC
3
than
for
p
62
.
In
contrast
,
TDP-
43
immunopositivity
was
highly
specific
for
IBM
,
but
the
sensitivity
of
this
test
was
low
,
with
definitive
staining
present
in
just
67
%
of
IBM
cases
.
To
differentiate
IBM
from
PM
,
we
thus
recommend
using
a
panel
of
LC
3
and
TDP-
43
antibodies
:
the
finding
of
<
14
%
LC
3
-
positive
fibers
helps
exclude
IBM
,
while
>
7
%
of
TDP-
43
-
positive
fibers
strongly
supports
a
diagnosis
of
IBM
.
These
data
provide
support
for
the
hypothesis
that
disruption
of
autophagy
and
protein
aggregation
contribute
to
IBM
pathogenesis
.
Diseases
Validation
Diseases presenting
"cell-mediated inflammatory myopathies"
symptom
inclusion body myositis
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