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Favorable radiological outcome of skeletal Erdheim-Chester disease involvement with anakinra.
[erdheim-chester disease]
Erdheim-
Chester
disease
is
a
rare
non-
langerhans
cell
histiocytosis
characterized
by
infiltration
of
foamy
CD
68
-
positive
but
CD
1
a-negative
macrophages
and
fibro-
inflammatory
lesions
as
retroperitoneal
,
periureteral
areas
or
bones
.
Interferon-α
therapy
has
been
used
as
treatment
but
it
had
variable
efficiency
and
limited
tolerance
.
More
recently
,
a
recombinant
form
of
interleukin-
1
receptor
antagonist
(
anakinra
)
was
used
with
success
but
no
skeletal
radiological
improvement
was
recorded
.
We
report
here
a
case
of
interleukin-
1
receptor
antagonist
in
the
treatment
of
refractory
bones
infiltration
in
Erdheim-
Chester
disease
.
After
1
year
of
treatment
,
the
positron
emission
tomography-computed
tomography
showed
an
outstanding
response
of
the
skeletal
involvement
with
clearly
lower
and
smaller
hypermetabolism
images
.
