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Cystic lung disease: achieving a radiologic diagnosis.
[lymphangioleiomyomatosis]
Diffuse
cystic
lung
disease
represents
a
diverse
group
of
uncommon
disorders
with
characteristic
appearance
on
high
resolution
CT
imaging
.
The
combination
of
imaging
appearance
with
clinical
features
and
genetic
testing
where
appropriate
permits
a
confident
and
accurate
diagnosis
in
the
majority
of
the
diseases
without
recourse
for
open
lung
biopsy
.
The
mechanism
of
cyst
development
disease
is
unclear
but
in
some
disorders
appears
to
be
related
to
small
airways
obstruction
.
These
diseases
are
incurable
,
with
the
exception
of
Langerhans
cell
histiocytosis
which
may
spontaneously
remit
or
resolve
on
smoking
cessation
.
Disease
progression
is
unpredictable
;
in
general
older
patients
have
a
more
benign
disease
,
while
young
patients
may
progress
rapidly
to
respiratory
failure
.
An
understanding
of
the
complications
of
cystic
lung
disease
and
the
appearance
of
disease
progression
is
essential
for
the
management
of
these
patients
.
A
number
of
these
disorders
are
associated
with
malignancy
,
recognition
of
the
potential
tumors
permits
appropriate
imaging
surveillance
.
Due
to
the
widespread
use
of
CT
,
pulmonary
cysts
are
increasingly
discovered
incidentally
in
an
asymptomatic
individual
.
The
diagnostic
challenge
is
to
determine
whether
these
cysts
represent
an
early
feature
of
a
progressive
disease
or
have
no
clinical
significance
.
In
the
elderly
population
the
cysts
are
unlikely
to
represent
a
progressive
disease
.
In
individuals
<
50
years
further
evaluation
is
recommended
.
Diseases
Validation
Diseases presenting
"the appearance of disease progression is essential for the management of these patients"
symptom
lymphangioleiomyomatosis
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