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[Inclusion-body myositis].
[inclusion body myositis]
Sporadic
inclusion-body
myositis
(
sIBM
)
presents
in
average
at
the
sixth
decade
of
life
and
affects
three
men
for
one
woman
.
It
is
a
non-
lethal
,
slowly
progressive
but
disabling
disease
.
Except
the
striated
muscles
,
no
other
organs
(
such
as
the
interstitial
lung
)
are
involved
.
The
phenotype
of
this
myopathy
is
particular
since
it
involves
the
axial
muscles
(
camptocormia
,
swallowing
dysfunction
)
and
limb
girdle
(
notably
the
quadriceps
)
but
also
the
distal
muscles
(
in
particular
the
fingers
'
and
wrists
'
flexors
)
in
a
bilateral
but
non-
symmetrical
manner
.
The
clinical
presentation
is
then
very
suggestive
of
the
diagnosis
,
which
remains
to
be
proven
by
a
muscle
biopsy
.
Histological
features
defining
the
diagnosis
associate
endomysial
inflammatory
infiltrates
with
frequent
invaded
fibres
(
the
myositis
)
and
amyloid
deposits
generally
accompanying
rimmed
vacuoles
(
the
inclusions
)
.
There
is
still
today
a
debate
to
know
if
this
disease
is
at
its
beginning
a
degenerative
or
an
auto-immune
condition
.
Nonetheless
,
usual
immunosuppressive
drugs
(
corticosteroids
,
azathioprine
,
methotrexate
)
or
polyvalent
immunoglobulines
remain
ineffective
and
even
may
worsen
the
handicap
.
Some
controlled
randomized
trials
will
soon
be
launched
for
this
condition
,
but
for
now
,
the
best
therapeutic
approach
to
slow
down
the
rapidity
of
progression
of
the
disease
is
to
maintain
muscle
exercise
with
the
help
of
the
physiotherapists
.
Diseases
Validation
Diseases presenting
"muscle biopsy"
symptom
cadasil
canavan disease
cohen syndrome
epidermolysis bullosa simplex
familial mediterranean fever
focal myositis
inclusion body myositis
neuralgic amyotrophy
pyomyositis
pyruvate dehydrogenase deficiency
sneddon syndrome
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