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[Recurrent pericardial effusion as first manifestation of Erdheim-Chester disease].
[erdheim-chester disease]
A
65
-
year
-old
woman
presented
with
reduced
general
condition
and
dyspnoea
that
was
progressive
over
the
last
months
.
Clinical
findings
revealed
an
exophthalmus
on
the
right
,
xanthelasm
and
mild
peripheral
oedema
.
Previously
,
a
pericardiocentesis
had
been
performed
due
to
a
large
pericardial
effusion
.
A
previous
CT
scan
showed
a
mass
attached
to
the
pericardium
extending
through
the
atrio-
ventricular
groove
and
a
thickened
aorta
.
In
addition
,
a
retroperitoneal
fibrosis
and
an
occlusion
of
both
Aa
.
iliacae
internae
were
found
.
The
ECG
showed
sinus
rhythm
.
Laboratory
findings
demonstrated
a
microcytic
anemia
and
a
renal
failure
.
Chest
radiography
showed
a
large
cardiac
silhouette
,
while
the
transthoracic
echocardiography
revealed
a
recurrent
large
pericardial
effusion
.
A
PET
/
CT
scan
of
the
chest
and
abdomen
showed
a
tissue
infiltration
of
the
retroperitoneal
structures
,
a
mass
surrounding
the
right
coronary
artery
and
the
right
orbita
.
Finally
,
a
femur
biopsy
confirmed
the
diagnosis
of
Erdheim-
Chester
disease
.
With
the
diagnosis
Erdheim-
Chester
disease
we
started
a
high
dose
immunsuppressive
therapy
using
glucocorticoids
and
interferon-a
.
Tumour
size
slightly
decreased
during
the
following
2
months
,
however
the
patient
developed
a
severe
urosepsis
and
died
from
multiorgan
failure
.
We
report
a
case
of
an
Erdheim-
Chester
disease
with
cardiovascular
involvement
primarily
diagnosed
due
to
a
recurrent
large
pericardial
effusion
.
In
case
of
cardial
tumors
with
interatrial
septum
or
coronary
artery
involvement
together
with
cerebral
manifestations
,
an
Erdheim-
Chester
disease
should
be
taken
into
account
.
Diseases
Validation
Diseases presenting
"a recurrent large pericardial effusion"
symptom
erdheim-chester disease
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