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Anti-IL-1 treatment in familial Mediterranean fever and related amyloidosis.
[familial mediterranean fever]
Colchicine
is
the
standard
treatment
in
familial
Mediterranean
fever
(
FMF
)
patients
.
New
treatment
strategies
are
needed
in
FMF
patients
who
were
unresponsive
to
colchicine
therapy
or
who
had
developed
amyloidosis
.
The
aim
of
this
study
was
to
present
clinical-laboratory
features
and
treatment
responses
of
pediatric
FMF
patients
that
were
treated
with
anti-
IL
-
1
therapies
.
Files
of
patients
who
had
been
followed
in
our
department
with
diagnosis
of
FMF
were
retrospectively
evaluated
.
Patients
that
have
been
receiving
anti-
IL
-
1
therapies
(
anakinra
or
canakinumab
)
were
included
to
the
study
.
All
patients
were
interpreted
with
respect
to
the
demographic
data
,
clinical
and
laboratory
features
of
the
disease
,
genetic
analysis
of
MEFV
mutations
and
treatment
responses
.
Among
330
currently
registered
FMF
patients
,
13
patients
were
included
to
the
study
.
Seven
of
them
received
anti-
IL
-
1
therapy
due
to
colchicine
resistance
and
6
due
to
FMF
-related
amyloidosis
(
1
of
them
with
nephrotic
syndrome
,
2
with
chronic
kidney
disease
,
3
with
renal
transplantation
)
.
In
all
treated
patients
,
attacks
completely
disappeared
or
decreased
in
frequency
;
partial
remission
occured
in
nephrotic
syndrome
patient
;
and
their
life
quality
improved
.
Anti-
IL
-
1
therapies
can
be
successfully
used
in
colchicine-resistant
FMF
patients
and
patients
with
amyloidosis
during
childhood
and
adolescent
period
without
major
side
effects
.
