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[Idiopathic inflammatory myopathies from the viewpoint of a neurologist].
[inclusion body myositis]
Idiopathic
inflammatory
myopathies
(
IIMs
)
are
a
heterogeneous
group
of
systemic
autoimmune
disorders
characterized
by
inflammation
of
skeletal
muscle
.
In
Japan
,
patients
with
IIMs
usually
visit
a
dermatologist
,
rheumatologist
,
or
neurologist
depending
on
the
main
symptom
.
Because
most
of
the
patients
with
IIMs
have
muscle
weakness
as
a
main
symptom
,
muscle
biopsy
is
usually
performed
to
differentiate
these
from
other
non-
inflammatory
myopathies
.
Thus
,
neurologists
in
Japan
tend
to
consider
mostly
the
pathological
findings
in
the
diagnosis
and
classification
of
IIMs
.
From
this
background
,
IIMs
have
been
classified
into
four
pathologically
distinct
subsets
:
polymyositis
,
dermatomyositis
,
necrotizing
autoimmune
myositis
,
and
sporadic
inclusion
body
myositis
.
However
,
in
clinical
practice
,
the
percentage
of
patients
with
typical
pathological
findings
is
generally
not
high
.
Therefore
,
other
clinical
factors
,
including
rash
or
clinical
complications
(
malignancy
,
collagen
diseases
,
or
interstitial
pneumonitis
)
,
have
been
used
along
with
pathological
classification
.
With
the
recent
discovery
of
new
myositis
-
specific
autoantibodies
(
MSAs
)
by
rheumatologists
and
dermatologists
,
it
has
been
suggested
that
the
presence
of
a
MSA
is
another
important
factor
for
classification
.
To
develop
useful
methods
of
classification
and
to
reveal
the
pathological
mechanisms
of
IIMs
,
further
collaborative
studies
by
dermatologists
,
rheumatologists
,
and
neurologists
are
necessary
.
Diseases
Validation
Diseases presenting
"inflammatory myopathies"
symptom
familial mediterranean fever
focal myositis
inclusion body myositis
pyomyositis
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