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[Sporadic inclusion body myositis and amyloid].
[inclusion body myositis]
Sporadic
inclusion
body
myositis
(
sIBM
)
is
an
intractable
and
progressive
skeletal
muscle
disease
of
unknown
etiology
and
without
effective
treatment
.
While
the
etiology
is
still
unknown
,
however
,
genetic
factors
,
aging
,
life
style
,
and
environmental
factors
may
be
involved
.
Muscle
biopsy
typically
reveals
endomysial
inflammation
,
invasion
of
mononuclear
cells
into
non-necrotic
fibers
and
rimmed
vacuoles
,
suggesting
inflammation
and
degeneration
co
-exist
as
part
of
the
pathomechanism
.
Recent
studies
implicate
amyloid
beta
accumulation
,
defects
of
proteolysis
,
and
immune
system
abnormalities
.
The
clinical
course
is
slow
with
chronic
worsening
.
Diagnosis
of
sIBM
is
usually
made
5
years
after
onset
.
Muscle
weakness
and
atrophy
in
the
quadriceps
,
wrist
flexor
and
finger
flexors
are
the
typical
neurological
findings
of
sIBM
.
Dysphagia
and
asymmetric
weakness
are
often
found
.
Serum
creatine
kinase
is
usually
below
2
,
000
IU
/
L
.
sIBM
is
generally
refractory
to
current
therapy
,
such
as
steroids
or
immunosuppressants
.
Elucidation
of
the
pathomechanism
of
sIBM
is
the
most
important
to
therapy
.
Diseases
Validation
Diseases presenting
"atrophy in the quadriceps"
symptom
inclusion body myositis
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