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Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation?
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
(
LAM
)
is
a
rare
cystic
lung
disease
characterized
by
proliferation
of
smooth
muscle
like
cells
(
LAM
cells
)
that
have
mutations
in
the
tuberous
sclerosis
gene
(
TSC
2
)
,
leading
to
the
activation
of
the
mammalian
target
of
rapamycin
(
mTOR
)
.
Rapamycin
,
an
inhibitor
of
the
mTOR
pathway
,
has
been
shown
in
a
landmark
clinical
trial
to
halt
the
decline
in
lung
function
,
as
long
as
it
is
used
continuously
.
Women
with
severe
pulmonary
LAM
still
progress
to
require
lung
transplantation
.
The
use
of
inhibitors
of
the
mTOR
pathway
immediately
after
transplant
has
been
linked
to
bronchial
anastomotic
dehiscence
,
a
potentially
fatal
complication
of
lung
transplantation
.
Currently
,
it
is
recommended
that
women
with
LAM
stop
taking
rapamycin
once
listed
for
lung
transplant
,
which
could
potentially
lead
to
faster
lung
function
decline
while
awaiting
organ
transplantation
.
Here
we
review
the
existing
evidence
and
discuss
potential
recommendations
for
the
management
of
the
inhibitors
of
the
mTOR
pathway
while
awaiting
lung
transplantation
.
Diseases
Validation
Diseases presenting
"potentially fatal complication"
symptom
heparin-induced thrombocytopenia
lymphangioleiomyomatosis
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