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Efficacy and safety of sirolimus for renal angiomyolipoma in patients with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis: a systematic review.
[lymphangioleiomyomatosis]
We
evaluate
the
efficacy
and
safety
of
sirolimus
in
the
treatment
of
renal
angiomyolipoma
in
patients
with
tuberous
sclerosis
complex
or
sporadic
lymphangioleiomyomatosis
.
A
systematic
search
of
MEDLINE
®
,
Embase
®
,
ACP
(
American
College
of
Physicians
)
Journal
Club
,
Cochrane
CENTRAL
(
Central
Register
of
Controlled
Trials
)
and
Cochrane
Database
of
Systematic
Reviews
was
performed
.
A
secondary
hand
search
was
performed
in
relevant
journals
,
references
and
the
grey
literature
.
The
screening
,
quality
assessment
and
data
extraction
of
the
retrieved
articles
were
independently
performed
by
2
reviewers
in
duplicate
.
Studies
that
reported
an
angiomyolipoma
response
or
adverse
events
after
the
treatment
of
sirolimus
were
included
in
the
analysis
.
Four
prospective
nonrandomized
studies
involving
94
patients
were
included
in
the
study
.
The
overall
response
rate
of
angiomyolipoma
was
46
.
8
%
(
44
of
94
)
in
the
first
year
.
In
the
second
year
the
angiomyolipoma
response
rate
for
those
patients
still
being
treated
with
sirolimus
was
43
.
5
%
(
20
of
46
)
and
the
response
rate
of
the
patients
whose
sirolimus
treatment
was
discontinued
was
5
%
(
2
of
40
)
.
The
most
common
sirolimus
related
adverse
reactions
were
stomatitis
,
respiratory
infection
,
skin
lesions
and
hyperlipidemia
,
while
serious
adverse
reactions
were
rarely
observed
.
This
study
shows
that
renal
angiomyolipoma
shrank
during
sirolimus
therapy
but
tended
to
regrow
after
the
therapy
was
stopped
.
In
general
,
sirolimus
is
an
effective
and
safe
therapy
for
renal
angiomyolipoma
in
patients
with
tuberous
sclerosis
complex
or
sporadic
lymphangioleiomyomatosis
.
