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[Renal involvement in Erdheim-Chester disease].
[erdheim-chester disease]
The
Erdheim-
Chester
disease
is
a
rare
form
of
Langherans
cells
.
Since
1987
it
is
distinguished
from
other
istiocytosis
previously
identified
.
The
diagnosis
of
the
disease
relies
on
defined
radiological
(
bone
imaging
)
and
pathological
(
histiocytic
infiltration
)
criteria
.
Bone
disease
is
crucial
but
systemic
manifestations
are
reported
more
frequently
at
onset
.
Renal
involvement
is
always
asymptomatic
at
onset
of
disease
or
in
the
follow-up
.
In
this
review
we
analyze
the
reports
of
the
literature
;
we
highlight
3
pathological
mechanisms
of
renal
involvement
:
renal
and
retroperitoneal
infiltration
,
urinary
tract
obstruction
,
renal
arteries
stenosis
.
No
treatment
to
date
has
demonstrated
an
improvement
in
survival
of
patients
with
EC
.
Renal
involvement
is
therefore
symptomatic
(
ureteral
stenting
,
percutaneous
nephrostomy
)
or
is
adopted
a
wait-and-see
attitude
.