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Biomarkers of inclusion body myositis.
[inclusion body myositis]
Inclusion
body
myositis
(
IBM
)
is
a
poorly
understood
autoimmune
and
degenerative
disorder
of
skeletal
muscle
.
Here
,
pathophysiological
and
diagnostic
biomarkers
of
IBM
are
reviewed
.
Muscle
histopathological
biomarkers
have
been
successful
in
stimulating
the
study
of
IBM
pathophysiology
for
over
three
decades
.
Their
use
as
diagnostic
biomarkers
,
in
contrast
,
has
significant
limitations
.
A
blood
biomarker
,
autoantibodies
against
a
43
-
kDa
muscle
protein
reported
in
2011
,
has
now
been
identified
as
targeting
cytoplasmic
5
'
nucleotidase
(
cN
1
A
;
NT
5
C
1
A
)
,
a
protein
involved
in
nucleic
acid
metabolism
.
Diagnostic
testing
for
these
autoantibodies
is
of
high
diagnostic
performance
for
IBM
.
Muscle
biomarkers
have
suggested
that
IBM
pathophysiology
is
linked
to
myonuclear
degeneration
and
disordered
nucleic
acid
metabolism
.
A
blood
biomarker
has
high
diagnostic
performance
for
IBM
,
and
through
identification
of
its
target
links
,
IBM
autoimmunity
and
degeneration
together
,
supporting
the
view
that
IBM
pathophysiology
includes
abnormal
nucleic
acid
metabolism
.
Diseases
Validation
Diseases presenting
"skeletal muscle"
symptom
cadasil
epidermolysis bullosa simplex
esophageal adenocarcinoma
focal myositis
hereditary cerebral hemorrhage with amyloidosis
inclusion body myositis
lamellar ichthyosis
neuralgic amyotrophy
pleomorphic liposarcoma
pyomyositis
pyruvate dehydrogenase deficiency
systemic capillary leak syndrome
well-differentiated liposarcoma
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