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Pulmonary lymphangioleiomyomatosis: literature update.
[lymphangioleiomyomatosis]
Pulmonary
lymphangioleiomyomatosis
is
an
uncommon
disease
of
unknown
etiology
characterized
by
the
proliferation
of
abnormal
smooth
muscle
cells
in
the
lungs
,
leading
to
parenchymal
destruction
and
progressive
respiratory
failure
.
The
natural
history
of
this
disease
remains
poorly
understood
,
primarily
seen
in
women
of
childbearing
age
.
The
diagnosis
can
be
difficult
because
symptoms
are
nonspecific
and
very
similar
to
other
respiratory
diseases
like
asthma
,
emphysema
and
bronchitis
.
Lymphangioleiomyomatosis
may
not
be
diagnosed
until
a
pneumothorax
,
chylothorax
,
interstitial
lung
disease
or
angiomyolipomas
are
discovered
.
The
recent
advances
in
genetic
and
molecular
research
provide
new
hope
to
discover
the
intricate
mechanism
of
disease
and
evaluate
new
therapies
.
Internists
,
primary
care
physicians
and
pulmonologists
should
be
aware
of
this
condition
in
order
to
avoid
delay
in
the
diagnosis
and
institute
appropriate
therapy
.
The
clinical
features
,
pathophysiology
,
molecular
genetics
and
medical
treatment
will
be
reviewed
.