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A retrospective cohort study identifying the principal pathological features useful in the diagnosis of inclusion body myositis.
[inclusion body myositis]
The
current
pathological
diagnostic
criteria
for
sporadic
inclusion
body
myositis
(
IBM
)
lack
sensitivity
.
Using
immunohistochemical
techniques
abnormal
protein
aggregates
have
been
identified
in
IBM
,
including
some
associated
with
neurodegenerative
disorders
.
Our
objective
was
to
investigate
the
diagnostic
utility
of
a
number
of
markers
of
protein
aggregates
together
with
mitochondrial
and
inflammatory
changes
in
IBM
.
Retrospective
cohort
study
.
The
sensitivity
of
pathological
features
was
evaluated
in
cases
of
Griggs
definite
IBM
.
The
diagnostic
potential
of
the
most
reliable
features
was
then
assessed
in
clinically
typical
IBM
with
rimmed
vacuoles
(
n
=
15
)
,
clinically
typical
IBM
without
rimmed
vacuoles
(
n
=
9
)
and
IBM
mimics-protein
accumulation
myopathies
containing
rimmed
vacuoles
(
n
=
7
)
and
steroid-responsive
inflammatory
myopathies
(
n
=
11
)
.
Specialist
muscle
services
at
the
John
Radcliffe
Hospital
,
Oxford
and
the
National
Hospital
for
Neurology
and
Neurosurgery
,
London
.
Individual
pathological
features
,
in
isolation
,
lacked
sensitivity
and
specificity
.
However
,
the
morphology
and
distribution
of
p
62
aggregates
in
IBM
were
characteristic
and
in
a
myopathy
with
rimmed
vacuoles
,
the
combination
of
characteristic
p
62
aggregates
and
increased
sarcolemmal
and
internal
major
histocompatibility
complex
class
I
expression
or
endomysial
T
cells
were
diagnostic
for
IBM
with
a
sensitivity
of
93
%
and
specificity
of
100
%
.
In
an
inflammatory
myopathy
lacking
rimmed
vacuoles
,
the
presence
of
mitochondrial
changes
was
100
%
sensitive
and
73
%
specific
for
IBM
;
characteristic
p
62
aggregates
were
specific
(
91
%
)
,
but
lacked
sensitivity
(
44
%
)
.
We
propose
an
easily
applied
diagnostic
algorithm
for
the
pathological
diagnosis
of
IBM
.
Additionally
our
findings
support
the
hypothesis
that
many
of
the
pathological
features
considered
typical
of
IBM
develop
later
in
the
disease
,
explaining
their
poor
sensitivity
at
disease
presentation
and
emphasising
the
need
for
revised
pathological
criteria
to
supplement
the
clinical
criteria
in
the
diagnosis
of
IBM
.
Diseases
Validation
Diseases presenting
"myositis"
symptom
focal myositis
inclusion body myositis
malignant atrophic papulosis
pyomyositis
systemic capillary leak syndrome
This symptom has already been validated