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Sustained Effects of Sirolimus on Lung Function and Cystic Lung Lesions in Lymphangioleiomyomatosis.
[lymphangioleiomyomatosis]
Rationale
.
Sirolimus
therapy
stabilizes
lung
function
and
reduces
the
size
of
chylous
effusions
and
lymphangioleiomyomas
in
patients
with
lymphangioleiomyomatosis
.
Objectives
.
To
determine
whether
sirolimus
has
beneficial
effects
on
lung
function
,
cystic
areas
and
adjacent
lung
parenchyma
,
whether
these
effects
are
sustained
,
and
sirolimus
is
well
tolerated
by
patients
.
Methods
.
Lung
function
decline
over
time
,
lung
volume
occupied
by
cysts
(
cyst
score
)
,
and
lung
tissue
texture
in
the
vicinity
of
the
cysts
,
were
quantified
with
a
computer-aided
diagnosis
system
,
in
38
patients
.
Then
we
compared
cyst
scores
from
the
last
study
off
sirolimus
with
studies
done
on
sirolimus
therapy
.
In
12
patients
,
we
evaluated
rates
of
change
in
lung
function
and
cyst
scores
off
and
on
sirolimus
.
Measurements
and
results
.
Sirolimus
reduced
yearly
declines
in
FEV
1
(
-
2
.
3
±
0
.
1
versus
1
.
0
±
0
.
3
%
predicted
,
p
<
0
.
001
)
and
DLCO
(
-
2
.
6
±
0
.
1
versus
0
.
9
±
0
.
2
%
predicted
,
p
<
0
.
001
)
.
Cyst
scores
1
.
2
±
0
.
8
years
(
30
.
5
±
11
.
9
%
)
,
and
2
.
5
±
2
years
(
29
.
7
±
12
.
1
%
)
after
initiating
sirolimus
were
not
significantly
different
from
pre-treatment
values
(
28
.
4
±
12
.
5
%
)
.
In
12
patients
,
followed
for
five
years
,
a
significant
reduction
in
rates
of
yearly
decline
in
FEV
1
(
-
1
.
4
±
0
.
2
versus
0
.
3
±
0
.
4
%
predicted
,
p
=
0
.
025
)
was
observed
.
Analyses
of
104
computed
tomography
scans
showed
a
non-significant
(
p
=
0
.
23
)
reduction
in
yearly
rates
of
change
of
cyst
scores
(
1
.
8
±
0
.
2
versus
0
.
3
±
0
.
3
%
,
p
=
0
.
23
)
,
and
lung
texture
features
.
Despite
adverse
events
,
most
patients
were
able
to
continue
sirolimus
therapy
.
Conclusions
.
Sirolimus
therapy
slowed
down
lung
function
decline
and
increase
in
cystic
lesions
.
Most
patients
were
able
to
tolerate
sirolimus
therapy
.
Diseases
Validation
Diseases presenting
"reduces the size of chylous effusions and lymphangioleiomyomas in patients with lymphangioleiomyomatosis"
symptom
lymphangioleiomyomatosis
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