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Idiopathic inflammatory myopathies.
[inclusion body myositis]
Idiopathic
inflammatory
myopathies
(
IIMs
)
,
except
for
sporadic
inclusion
body
myositis
(
sIBM
)
,
present
with
subacute
symmetrical
weakness
of
the
limb
girdle
muscles
,
an
elevated
serum
creatine
kinase
activity
,
and
inflammatory
cells
in
the
muscle
biopsy
(
necrotizing
autoimmune
myopathy
being
an
exception
)
.
In
dermatomyositis
,
additional
skin
abnormalities
are
found
.
IIMs
are
nowadays
subclassified
into
the
following
categories
:
(
1
)
dermatomyositis
(
DM
)
,
including
(
1
a
)
classic
dermatomyositis
,
which
may
be
associated
with
connective
tissue
disorders
(
CTDs
)
and
malignancy
,
(
1
b
)
juvenile
dermatomyositis
,
and
(
1
c
)
clinical
amyopathic
dermatomyositis
;
(
2
)
polymyositis
(
PM
)
encompassing
(
2
a
)
classical
PM
and
(
2
b
)
nonspecific
or
overlap
myositis
,
associated
with
CTD
;
(
3
)
autoimmune
necrotizing
myopathy
,
associated
with
malignancy
,
statin
use
and
CTD
;
and
(
4
)
sporadic
IBM
,
sometimes
associated
with
CTDs
.
These
conditions
result
from
chronic
immune
activation
after
exposure
to
environmental
risk
factors
in
individuals
with
a
predisposing
genetic
background
.
A
strong
association
of
autoantibodies
with
distinct
clinical
phenotypes
and
prognosis
is
found
in
patients
with
myositis
.
Inflammatory
myopathies
,
sporadic
IBM
excluded
,
are
amenable
to
immunosuppressive
and
immunomodulation
therapies
.
The
prognosis
of
IIM
is
not
well
known
since
long
-term
outcome
and
prognostic
factors
vary
widely
.
Disease-related
mortality
rates
in
PM
and
DM
are
at
least
10
%
.
In
DM
mortality
is
attributed
to
cancer
and
pulmonary
complications
.
Juvenile
dermatomyositis
has
a
low
mortality
rate
.
Because
chronic
immunosuppressive
therapy
is
associated
with
significant
side-effects
,
and
many
patients
remain
(
partially
)
refractory
to
treatment
,
novel
therapeutic
agents
that
are
safe
and
effective
are
needed
.
Diseases
Validation
Diseases presenting
"clinical amyopathic dermatomyositis"
symptom
inclusion body myositis
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