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Pulmonary lymphangioleiomyomatosis: a rare case.
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
is
an
uncommon
lung
disease
primarily
affecting
women
of
childbearing
age
.
It
is
characterized
by
the
progressive
proliferation
and
infiltration
of
smooth
muscle
-like
cells
,
which
lead
to
cystic
destruction
of
the
lung
parenchyma
;
obstruction
of
airways
,
blood
vessels
,
and
lymphatics
;
and
loss
of
pulmonary
function
.
We
present
the
case
of
a
46
-
year
-old
female
patient
with
chest
pain
,
cough
,
sputum
,
and
dyspnea
on
exertion
for
three
weeks
.
Minimal
pneumothorax
was
noted
,
and
the
patient
was
referred
to
our
center
for
further
investigation
and
treatment
.
High
-resolution
computed
tomography
revealed
numerous
bilateral
thin
-walled
air
cysts
and
interstitial
thickening
affecting
the
central
and
peripheral
part
of
the
upper
zone
of
the
lung
.
We
performed
an
open
-
lung
biopsy
to
confirm
lymphangioleiomyomatosis
.
Our
aim
is
to
discuss
the
pathogenesis
and
other
lesions
noted
in
the
differential
diagnosis
of
this
rare
disease
.
Diseases
Validation
Diseases presenting
"progressive proliferation"
symptom
lymphangioleiomyomatosis
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