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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
"muscle weakness"
symptom
alexander disease
canavan disease
coats disease
cohen syndrome
cystinuria
familial mediterranean fever
focal myositis
inclusion body myositis
neuralgic amyotrophy
pyomyositis
severe combined immunodeficiency
systemic capillary leak syndrome
thoracic outlet syndrome
triple a syndrome
von hippel-lindau disease
This symptom has already been validated