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Clinical assessment determines the diagnosis of inclusion body myositis independently of pathological features.
[inclusion body myositis]
Historically
,
the
diagnosis
of
sporadic
inclusion
body
myositis
(
IBM
)
has
required
the
demonstration
of
the
presence
of
a
number
of
histopathological
findings
on
muscle
biopsy--namely
,
rimmed
vacuoles
,
an
inflammatory
infiltrate
with
invasion
of
non-necrotic
muscle
fibres
(
partial
invasion
)
and
amyloid
or
15
-
18
nm
tubulofilamentous
inclusions
(
Griggs
criteria
)
.
However
,
biopsies
of
many
patients
with
clinically
typical
IBM
do
not
show
all
of
these
histopathological
findings
,
at
least
at
presentation
.
We
compared
the
clinical
features
at
presentation
and
during
the
course
of
disease
in
67
patients
with
histopathologically
diagnosed
IBM
and
clinically
diagnosed
IBM
seen
within
a
single
UK
specialist
muscle
centre
.
At
presentation
,
using
clinically
focused
diagnostic
criteria
(
European
Neuromuscular
Centre
(
ENMC
)
2011
)
,
a
diagnosis
of
IBM
was
made
in
88
%
of
patients
whereas
76
%
fulfilled
the
1997
ENMC
criteria
and
only
27
%
satisfied
the
histopathologically
focused
Griggs
criteria
.
There
were
no
differences
in
clinical
features
or
outcomes
between
clinically
and
histopathologically
diagnosed
patients
,
but
patients
lacking
the
classical
histopathological
finding
of
rimmed
vacuoles
were
younger
,
suggesting
that
rimmed
vacuoles
may
be
a
later
feature
of
the
disease
.
These
findings
have
important
implications
for
diagnosis
and
future
studies
or
trials
in
IBM
as
adherence
to
histopathologically
focused
diagnostic
criteria
will
exclude
large
numbers
of
patients
with
IBM
.
Importantly
,
those
excluded
may
be
at
an
earlier
stage
of
the
disease
and
more
amenable
to
treatment
.
Diseases
Validation
Diseases presenting
"but patients lacking the classical histopathological finding of rimmed vacuoles were younger"
symptom
inclusion body myositis
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