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[Idiopathic inflammatory myopathies from the viewpoint of rheumatologists].
[inclusion body myositis]
Idiopathic
inflammatory
myopathies
(
IIMs
)
are
a
group
of
inflammatory
muscle
disorders
of
unknown
etiology
;
these
include
polymyositis
(
PM
)
,
dermatomyositis
(
DM
)
,
and
inclusion
body
myositis
.
Extra
-
muscular
manifestations
such
as
dermatitis
,
arthritis
,
interstitial
lung
disease
(
ILD
)
,
cardiomyopathy
,
and
enteropathy
are
occasional
complications
in
patients
with
PM
/
DM
.
Several
myositis
-
specific
autoantibodies
(
MSAs
)
have
been
discovered
in
IIMs
;
these
can
help
predict
clinical
characteristics
,
response
to
treatment
,
and
prognosis
.
For
example
,
anti-aminoacyl-t
RNA
synthetase
(
ARS
)
antibodies
,
including
Jo-
1
antibody
(
Ab
)
and
anti-
melanoma
differentiation-associated
gene
5
(
MDA-
5
)
Ab
,
have
been
associated
with
the
manifestation
of
ILD
in
PM
and
DM
.
Anti-
MDA
5
Ab
-associated
ILD
has
a
1
-
year
survival
rate
of
50
-
60
%
;
however
,
short
-term
prognosis
is
relatively
good
in
anti-
ARS
Ab
-associated
ILD
.
Fatal
outcome
occurs
remarkably
often
within
the
first
6
months
of
anti-
MDA
5
Ab
-associated
ILD
.
Therefore
,
intensive
treatment
should
be
administered
to
patients
harboring
anti-
MDA-
5
Ab
or
showing
hyperferritinemia
in
ILD
with
DM
.
In
addition
,
corticosteroid
occasionally
induces
myopathy
,
which
is
an
issue
arising
in
PM
/
DM
treatment
.
Some
experts
recommend
combination
therapy
of
corticosteroid
and
an
immunosuppressive
agent
as
a
first
-line
treatment
for
myositis
in
PM
/
DM
.
Methotrexate
and
azathioprine
are
commonly
used
immunosuppressive
agents
for
myositis
in
western
countries
.
Immunosuppressive
agents
are
steroid-sparing
,
serving
to
mitigate
corticosteroid-related
side
effects
,
thus
making
combination
therapy
an
effective
treatment
option
.
Preventing
the
progression
of
physical
dysfunction
is
of
prime
importance
to
patients
with
PM
/
DM
.
Dermatologists
,
neurologists
,
and
rheumatologists
should
therefore
work
together
to
care
for
these
patients
before
muscular
and
extra
-
muscular
involvement
develop
progressively
and
irreversibly
.
Diseases
Validation
Diseases presenting
"inflammatory myopathies"
symptom
familial mediterranean fever
focal myositis
inclusion body myositis
pyomyositis
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