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The effect of anakinra, an IL1 receptor antagonist, in patients with sporadic inclusion body myositis (sIBM): a small pilot study.
[inclusion body myositis]
In
sIBM
,
an
inflammatory
process
mediated
by
cytotoxic
T
cells
and
cytokines
in
conjunction
with
a
degenerative
process
,
deposits
of
beta
amyloid
and
misfolded
proteins
appear
to
be
the
main
culprits
in
disease
pathogenesis
.
IL
-
1
β
may
play
a
key
role
because
it
is
upregulated
in
sIBM
myofibers
,
co
-localizes
with
Amyloid
Precursor
Protein
(
APP
)
and
promotes
the
production
of
APP
and
amyloid
deposits
.
We
performed
a
small
,
pilot
study
to
examine
whether
anakinra
,
an
IL
1
receptor
antagonist
could
benefit
sIBM
patients
.
Four
patients
with
biopsy-proven
sIBM
received
anakinra
for
a
mean
period
of
7
.
7
months
.
No
improvement
in
muscle
strength
or
stabilization
was
noted
in
any
of
the
patients
based
on
grip
strength
and
MRC
measurements
.
The
treatment
failure
may
be
due
to
insufficiency
of
anakinra
to
suppress
the
intramuscular
IL
1
,
the
short
study
period
,
or
the
irrelevance
of
IL
1
in
the
disease
process
.