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Multi-country estimate of different manifestations of aspergillosis in cystic fibrosis.
[allergic bronchopulmonary aspergillosis]
Aspergillus
spp
.
can
lead
to
allergic
bronchopulmonary
aspergillosis
(
ABPA
)
,
Aspergillus
sensitisation
and
Aspergillus
bronchitis
in
CF
.
The
relative
frequencies
of
these
entities
have
recently
been
ascertained
in
a
large
UK
adult
CF
cohort
.
We
have
used
this
data
to
estimate
the
burden
of
aspergillosis
and
ABPA
cases
in
adult
CF
patients
in
30
countries
reporting
CF
.
National
and
international
CF
registry
data
was
accessed
and
assessed
for
completeness
and
age
distribution
.
Published
proportions
of
ABPA
(
17
.
7
%
)
,
Aspergillus
sensitisation
(
14
.
6
%
)
and
Aspergillus
bronchitis
(
30
%
)
in
CF
were
applied
to
those
>
18
years
and
compared
with
notified
ABPA
cases
.
Of
the
76
,
201
estimated
CF
patients
worldwide
(
not
including
India
)
,
37
,
714
were
>
18
years
.
The
proportion
of
adults
to
children
varied
from
63
%
in
Norway
to
20
%
in
Brazil
.
ABPA
caseload
in
adults
is
anticipated
to
be
6
,
675
cases
of
which
only
2
,
221
cases
(
33
%
)
are
currently
recorded
,
indicating
substantial
underdiagnosis
.
The
ABPA
diagnosis
rate
compared
with
estimated
rates
varies
by
country
from
101
%
(
France
)
to
14
.
5
%
(
Greece
)
,
although
genetic
variation
could
account
for
genuine
differences
compared
with
the
UK
.
Aspergillus
bronchitis
is
not
currently
recognised
or
recorded
in
CF
registries
but
there
are
an
anticipated
10
,
988
adult
cases
.
Aspergillus
sensitisation
,
associated
with
increased
bronchiectasis
and
reduced
FEV
1
,
affects
an
anticipated
5
,
506
patients
without
ABPA
or
Aspergillus
bronchitis
.
Together
ABPA
and
Aspergillus
bronchitis
are
estimated
to
affect
17
,
989
adults
,
47
.
7
%
of
the
adult
CF
population
.
ABPA
also
occurs
in
children
and
teenagers
and
984
cases
were
documented
in
registries
.
Diagnosed
ABPA
rates
by
age
were
available
for
the
ECFS
registry
,
USA
,
UK
,
Ireland
,
Belgium
and
Netherlands
.
The
rate
was
<
1
%
under
4
years
,
and
increased
throughout
childhood
and
adolescence
,
with
marked
variation
between
countries
.
Newly
published
diagnostic
criteria
and
methods
should
facilitate
better
recognition
of
aspergillosis
in
CF
,
allowing
better
CF
disease
control
.
Diseases
Validation
Diseases presenting
"methods should facilitate better recognition of aspergillosis in cf"
symptom
allergic bronchopulmonary aspergillosis
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