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Pulmonary hypertension in chronic interstitial lung diseases.
[lymphangioleiomyomatosis]
Pulmonary
hypertension
(
PH
)
is
a
common
complication
of
interstitial
lung
diseases
(
ILDs
)
,
particularly
in
idiopathic
pulmonary
fibrosis
and
ILD
associated
with
connective
tissue
disease
.
However
,
other
lung
diseases
,
such
as
combined
pulmonary
fibrosis
and
emphysema
syndrome
,
pulmonary
Langerhans
cell
histiocytosis
,
and
lymphangioleiomyomatosis
,
may
also
include
PH
in
their
clinical
manifestations
.
In
all
of
these
diseases
,
PH
is
associated
with
reduced
exercise
capacity
and
poor
prognosis
.
The
degree
of
PH
in
ILDs
is
typically
mild
-
to
-
moderate
.
However
,
some
of
these
patients
may
develop
a
disproportionate
increase
in
PH
that
can
not
be
justified
solely
by
hypoxia
and
parenchymal
injury
:
this
condition
has
been
termed
"
out-of-proportion
"
PH
.
The
pathogenesis
of
PH
in
these
diseases
is
various
,
incompletely
understood
and
may
be
multifactorial
.
The
clinical
suspicion
(
i
.
e
.
increased
dyspnoea
,
low
diffusion
capacity
)
and
echocardiographic
assessment
are
the
first
steps
towards
proper
diagnosis
of
PH
;
however
,
right
heart
catheterisation
remains
the
current
gold
standard
for
diagnosis
of
PH
.
At
present
,
no
specific
therapies
have
been
approved
for
the
treatment
of
PH
in
patients
with
ILDs
.
Diseases
Validation
Diseases presenting
"such as combined pulmonary fibrosis and emphysema syndrome"
symptom
lymphangioleiomyomatosis
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