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Ex vivo PBMC cytokine profile in familial Mediterranean fever patients: Involvement of IL-1β, IL-1α and Th17-associated cytokines and decrease of Th1 and Th2 cytokines.
[familial mediterranean fever]
In
order
to
clarify
the
inflammatory
mechanism
underlying
familial
Mediterranean
fever
(
FMF
)
,
we
aimed
to
evaluate
the
ex
vivo
cytokine
profile
of
FMF
patients
during
acute
attacks
and
attack-free
periods
,
and
compare
it
with
that
of
healthy
controls
.
The
study
included
34
FMF
patients
,
of
whom
9
were
studied
during
attack
and
remission
and
24
healthy
controls
.
Cytokine
levels
were
evaluated
by
Luminex
technology
in
serum
and
supernatants
of
PBMC
(
Peripheral
Blood
Mononuclear
Cells
)
cultures
with
and
without
24
h
stimulation
of
monocytes
by
LPS
and
T
lymphocytes
by
anti-
CD
3
/
CD
28
beads
.
Levels
of
IL
-
6
and
TNF
-α
were
higher
in
unstimulated
and
LPS-stimulated
PBMC
supernatants
of
FMF
patients
in
crises
compared
to
controls
.
In
response
to
LPS
stimulation
,
higher
levels
of
IL
-
1
β
and
IL
-
1
α
were
found
in
PBMC
supernatants
of
patients
during
crises
compared
to
those
in
remission
and
to
controls
.
IFN-γ
and
IL
-
4
levels
were
the
lowest
in
unstimulated
and
anti-
CD
3
/
CD
28
stimulated
PBMCs
supernatants
of
patients
during
crises
compared
to
remission
and
controls
.
The
Th
17
cytokines
IL
-
17
and
IL
-
22
were
respectively
higher
in
anti-
CD
3
/
CD
28
stimulated
PBMC
supernatants
of
FMF
patients
during
and
between
crises
compared
to
controls
.
Amongst
cytokines
tested
in
serum
,
only
IL
-
6
and
TNF
α
were
enhanced
in
FMF
patients
.
The
ex
vivo
study
represents
an
interesting
approach
to
evaluate
cytokines
'
involvement
in
FMF
.
Our
results
suggest
an
ongoing
subclinical
inflammation
and
define
an
elevated
inflammatory
cytokine
signature
,
distinctly
for
M
694
V
homozygous
patients
.
The
absence
of
spontaneous
IL
-
1
β
release
by
PBMCs
reflects
no
constitutive
activation
of
the
inflammasome
in
FMF
physiopathology
.
Diseases
Validation
Diseases presenting
"fever"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
alexander disease
allergic bronchopulmonary aspergillosis
canavan disease
carcinoma of the gallbladder
child syndrome
congenital toxoplasmosis
cushing syndrome
cystinuria
dracunculiasis
erdheim-chester disease
esophageal adenocarcinoma
esophageal carcinoma
familial mediterranean fever
focal myositis
hodgkin lymphoma, classical
lamellar ichthyosis
legionellosis
locked-in syndrome
malignant atrophic papulosis
neonatal adrenoleukodystrophy
neuralgic amyotrophy
oculocutaneous albinism
papillon-lefèvre syndrome
pyomyositis
pyruvate dehydrogenase deficiency
scrub typhus
severe combined immunodeficiency
sneddon syndrome
systemic capillary leak syndrome
triple a syndrome
typhoid
waldenström macroglobulinemia
wolf-hirschhorn syndrome
This symptom has already been validated