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Successful treatment of Erdheim-Chester disease with combination of interleukin-1-targeting drugs and high-dose glucocorticoids.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
histocytic
disorder
.
We
report
a
case
of
a
45
-
year
-old
male
ECD
patient
with
severe
clinical
manifestation
(
urinary
obstruction
due
to
retroperitoneal
mass
with
hydronephrosis
,
involvement
of
long
bones
)
and
central
nervous
system
involvement
(
hemiparesis
,
aphasia
and
diabetes
insipidus
)
.
Diagnosis
was
confirmed
by
typical
clinical
,
radiological
and
histological
findings
.
Under
immunosuppressive
therapy
with
prednisolone
and
interleukin-
1
A
receptor
antagonist
(
Anakinra
,
Kineret
,
Swedish
Orphan
Biovitrum
AB
,
Stockholm
,
Sweden
)
,
a
rapid
improvement
of
the
patients
'
symptoms
and
condition
was
observed
.
This
is
the
first
report
of
a
successful
combination
therapy
of
Anakinra
and
glucocorticoids
.
Furthermore
,
current
literature
about
ECD
and
treatment
options
are
discussed
.
