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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
"limb girdle"
symptom
coats disease
inclusion body myositis
liposarcoma
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