Rare Diseases Symptoms Automatic Extraction
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A random Abstract
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Our Team
Erdheim-Chester disease.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
,
non-
Langerhans
form
of
histiocytosis
first
described
in
1930
with
a
wide
range
of
manifestations
.
The
number
of
new
cases
has
dramatically
increased
over
the
past
10
years
because
of
the
better
recognition
of
this
condition
.
The
natural
evolution
is
variable
,
but
the
spontaneous
prognosis
is
severe
.
In
this
review
,
we
describe
the
relevant
clinical
,
radiological
,
prognostic
,
and
therapeutic
features
of
this
orphan
disease
.
Compelling
evidence
demonstrates
the
efficacy
of
treatment
by
interferon
alpha
(
IFN
α
)
which
has
been
reported
to
be
a
major
independent
predictor
of
survival
among
ECD
patients
.
Alternative
treatments
remain
to
be
defined
.
Recent
studies
have
highlighted
the
central
nervous
system
involvement
as
an
independent
predictor
of
death
.
Pathophysiology
is
better
understood
with
a
complex
network
of
cytokines
and
chemokines
and
a
systemic
immune
Th-
1
-
oriented
perturbation
.
ECD
,
although
a
rare
and
orphan
disease
,
has
been
overlooked
and
numerous
new
cases
are
currently
diagnosed
because
of
general
better
knowledge
of
this
histiocytosis
.
First
-line
treatment
is
IFN
α
.
We
have
recently
described
a
unique
cytokine
signature
that
may
provide
further
clues
to
understand
the
pathogenesis
of
ECD
,
as
well
as
provide
new
tools
for
diagnosis
and
targeted
therapy
.
