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[Interleukin-1 receptor blockade with anakinra provided cessation of fatigue, reduction in inflammation markers and regression of retroperitoneal fibrosis in a patient with Erdheim-Chester disease - case study and a review of literature].
[erdheim-chester disease]
We
describe
a
case
of
an
Erdheim-
Chester
disease
patient
.
First
line
chemotherapy
treatment
with
2
-
chlorodeoxyadenosine
did
not
reduce
fluorodeoxyglucose
accumulation
in
pathological
lesions
.
The
patient
had
continuously
increased
CRP
values
of
17
-
20
mg
/
l
.
The
disease
continued
to
cause
subfebrile
temperatures
and
significant
fatigue
that
made
the
patient
to
spend
most
of
the
daytime
in
bed
.
To
manage
the
permanently
increased
inflammation
markers
,
we
decided
to
start
treatment
with
anakinra
,
successfully
used
in
some
other
autoinflammatory
diseases
(
e
.
g
.
Schnitzler
syndrome
)
.
We
have
now
been
able
to
evaluate
the
first
6
months
of
treatment
.
Daily
subcutaneous
administration
of
anakinra
(
KineretTM
100
mg
daily
)
led
to
normalization
of
CRP
values
,
cessation
of
subfebrile
temperatures
and
,
importantly
,
significant
reduction
of
fatigue
.
Time
periods
the
patient
was
able
to
spend
out
of
the
bed
increased
significantly
.
Consequent
to
the
reduced
fatigue
,
the
patient
was
able
to
perform
basic
household
tasks
he
was
unable
to
undertake
without
treatment
.
After
3
months
of
treatment
,
fatigue
of
the
same
intensity
returned
following
a
short
interruption
of
therapy
.
The
CRP
values
went
up
again
to
12
mg
/
l
.
CRP
value
returned
back
to
norm
and
fatigue
ceased
after
re
-initiation
of
daily
Kineret
injections
.
Objective
treatment
response
was
assessed
by
measuring
the
degree
of
fluorodeoxyglucose
accumulation
in
pathological
bone
lesions
.
PET-
CT
was
performed
before
and
3
and
6
months
after
anakinra
initiation
.
Intensity
of
accumulation
did
not
change
significantly
after
the
first
3
months
of
therapy
but
decreased
after
6
month
therapy
.
Follow
up
CT
of
abdominal
cavity
was
performed
at
the
end
of
the
6
th
month
of
treatment
.
Presented
CT
images
from
before
and
6
months
after
the
treatment
evidence
an
obvious
reduction
in
fibroid
changes
in
the
retroperitoneum
.
Daily
administration
of
anakinra
to
a
patient
with
active
Erdheim-
Chester
disease
significantly
reduced
intensity
of
fatigue
and
improved
quality
of
life
,
led
to
a
reduction
in
inflammatory
markers
and
regression
in
retroperitoneal
fibrotization
.
Diseases
Validation
Diseases presenting
"bone lesions"
symptom
erdheim-chester disease
familial hypocalciuric hypercalcemia
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