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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
"myopathy"
symptom
coats disease
cushing syndrome
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
familial mediterranean fever
focal myositis
homocystinuria without methylmalonic aciduria
inclusion body myositis
junctional epidermolysis bullosa
lymphangioleiomyomatosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
pyruvate dehydrogenase deficiency
This symptom has already been validated