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A cohort study of mortality predictors in patients with acute exacerbation of chronic fibrosing interstitial pneumonia.
[lymphangioleiomyomatosis]
To
assess
clinical
,
laboratory
and
radiographic
findings
associated
with
outcomes
and
to
clarify
more
practical
ways
to
predict
hospital
mortality
in
patients
with
acute
exacerbation
(
AE
)
of
chronic
fibrosing
interstitial
pneumonia
(
CFIP
)
.
Single
-centre
retrospective
cohort
study
.
University
Hospital
in
Japan
.
We
identified
51
consecutive
patients
with
AE
of
idiopathic
CFIP
through
multidisciplinary
discussion
.
Patients
who
had
connective
tissue
disease
,
drug-induced
lung
disease
,
pneumoconiosis
,
hypersensitivity
pneumonitis
,
sarcoidosis
,
pulmonary
histiocytosis
,
lymphangioleiomyomatosis
and
eosinophilic
pneumonia
were
excluded
.
There
were
no
interventions
.
The
main
outcome
was
determination
of
in
-hospital
mortality
predictors
.
Other
outcomes
included
clinical
,
laboratory
and
radiographic
differences
between
non-survivors
and
survivors
in
patients
with
AE
of
CFIP
.
T
he
mean
age
of
the
patients
with
AE
of
CFIP
was
71
years
.
Compared
with
survivors
,
non-survivors
had
a
significantly
shorter
duration
of
symptoms
before
admission
,
lower
prevalence
of
peripheral
distribution
of
ground-glass
opacity
and
centrilobular
emphysema
(
CLE
)
on
thin
-section
CT
,
lower
peripheral
lymphocyte
count
,
higher
brain
natriuretic
peptide
titre
,
lower
Pao
2
:
Fio
2
(
P
:
F
)
ratio
,
higher
prevalence
of
systemic
inflammatory
response
syndrome
(
SIRS
)
and
higher
SIRS
score
on
admission
(
p
=
0
.
0069
,
0
.
0032
,
0
.
015
,
0
.
040
,
0
.
0098
,
0
.
012
,
9
.
9
×
10
(
-
7
)
and
5
.
4
×
10
(
-
6
)
,
respectively
)
.
Multivariate
analysis
revealed
SIRS
(
HR
=
6
.
2810
,
p
=
0
.
015
)
,
CLE
(
HR
=
0
.
0606
,
p
=
3
.
6
×
10
(
-
5
)
)
and
serum
procalcitonin
level
(
HR
=
2
.
7110
,
p
=
0
.
022
)
to
be
independent
predictors
of
in
-hospital
mortality
.
A
Kaplan-
Meier
estimate
on
the
basis
of
stratification
according
to
the
presence
or
absence
of
SIRS
and
CLE
demonstrated
a
distinct
survival
curve
for
each
subset
of
patients
.
Distinct
survival
curves
documented
by
stratification
according
to
the
presence
or
absence
of
SIRS
and
CLE
may
provide
basic
information
for
a
rational
management
strategy
for
patients
with
AE
of
CFIP
on
admission
.
Diseases
Validation
Diseases presenting
"single-centre retrospective cohort study"
symptom
lymphangioleiomyomatosis
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