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FMF50: a score for assessing outcome in familial Mediterranean fever.
[familial mediterranean fever]
Colchicine
is
the
main
treatment
for
familial
Mediterranean
fever
(
FMF
)
.
However
,
biological
agents
and
other
treatments
are
available
for
patients
who
are
unable
to
receive
optimal
treatment
.
To
develop
outcome
criteria
that
define
response
to
treatment
.
Two
rounds
of
Delphi
exercise
were
followed
by
a
consensus
conference
enabling
the
definition
of
the
criteria
to
be
employed
.
Data
for
patients
with
FMF
responding
and
resistant
to
their
treatment
were
obtained
from
the
FMF
Arthritis
Vasculitis
and
Orphan
disease
Research
in
paediatric
rheumatology
(
FAVOR
)
website
.
The
suggested
criteria
were
analysed
and
validated
in
this
patient
cohort
.
Sensitivity
/
specificity
measures
and
the
ability
of
the
score
to
discriminate
between
patients
with
active
and
inactive
disease
via
the
best
cut-off
score
were
calculated
by
a
receiver
operating
characteristic
analysis
.
Compliance
with
the
maximum
dose
of
the
drug
was
considered
essential
for
evaluation
of
the
patients
.
Seven
criteria
were
suggested
in
the
consensus
conference
.
The
performance
of
each
criterion
,
in
differentiating
between
resistant
and
responsive
patients
,
was
tested
.
The
final
set
of
criteria
was
defined
as
at
least
50
%
improvement
in
five
of
six
criteria
,
without
worsening
in
any
one
defined
response
to
treatment
with
a
very
high
sensitivity
and
specificity
.
The
items
of
this
FMF
50
included
:
1
.
Percentage
change
in
the
frequency
of
attacks
with
the
treatment
.
2
.
Percentage
change
in
the
duration
of
attacks
with
the
treatment
.
3
.
Patients
/
parents
'
global
assessment
of
disease
severity
(
10
cm
visual
analogue
scale
(
VAS
)
)
.
4
.
Physicians
'
global
assessment
of
disease
severity
(
10
cm
VAS
)
.
5
.
Percentage
change
in
arthritis
attacks
with
the
treatment
.
6
.
Percentage
change
in
C-
reactive
protein
,
erythrocyte
sedimentation
rate
or
serum
amyloid
A
level
with
the
treatment
.
The
FMF
50
produced
is
a
user-friendly
measurement
tool
to
guide
physicians
and
can
be
used
in
clinical
trials
.
Diseases
Validation
Diseases presenting
"specificity"
symptom
familial mediterranean fever
hodgkin lymphoma, classical
inclusion body myositis
pyruvate dehydrogenase deficiency
typhoid
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