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Sporadic inclusion body myositis: new insights and potential therapy.
[inclusion body myositis]
To
describe
new
insights
and
developments
in
the
pathogenesis
,
diagnosis
and
treatment
of
sporadic
inclusion
body
myositis
(
IBM
)
.
Various
hypothesis
about
the
pathogenesis
of
IBM
continue
to
be
investigated
,
including
autoimmune
factors
,
mitochondrial
dysfunction
,
protein
dyshomeostasis
,
altered
nucleic
acid
metabolism
,
myonuclear
degeneration
and
the
role
of
the
myostatin
pathway
.
Serum
autoantibodies
against
cytosolic
5
'
-
nucleotidase
1
A
have
been
identified
in
IBM
showing
moderate
diagnostic
performance
.
The
differential
diagnostic
value
of
histopathological
features
,
including
different
protein
aggregates
,
continues
to
be
evaluated
.
MRI
may
also
be
of
monitoring
value
in
IBM
.
New
therapeutic
strategies
are
being
tested
in
IBM
patients
,
namely
the
upregulation
of
the
heat
shock
response
and
the
antagonism
of
myostatin
.
Recent
important
advances
have
occurred
in
IBM
.
These
advances
,
including
recent
and
ongoing
clinical
trials
,
may
lead
to
earlier
diagnosis
and
improved
understanding
and
treatment
of
the
disease
.
Despite
improved
knowledge
,
IBM
continues
to
be
a
puzzling
disease
and
the
pathogenesis
remains
to
be
clarified
.
An
interdisciplinary
,
bench
to
bedside
translational
research
approach
is
crucial
for
the
successful
identification
of
novel
treatments
for
this
debilitating
,
currently
untreatable
disorder
.
Diseases
Validation
Diseases presenting
"myositis"
symptom
focal myositis
inclusion body myositis
malignant atrophic papulosis
pyomyositis
systemic capillary leak syndrome
This symptom has already been validated