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Exertional leg pain in familial mediterranean Fever: a manifestation of an underlying enthesopathy and a marker of more severe disease.
[familial mediterranean fever]
Exertional
leg
pain
is
a
characteristic
musculoskeletal
manifestation
of
familial
Mediterranean
fever
(
FMF
)
.
We
aimed
to
define
the
frequency
and
characteristics
of
exertional
leg
pain
in
a
large
cohort
of
FMF
patients
and
to
evaluate
for
additional
signs
and
symptoms
of
spondyloarthritis
(
SpA
)
in
this
patient
population
.
FMF
patients
were
allocated
into
study
or
control
groups
based
on
the
presence
or
absence
of
exertional
leg
pain
.
Randomly
selected
patients
underwent
magnetic
resonance
imaging
(
MRI
)
of
the
ankle
as
well
as
plain
radiography
of
the
sacroiliac
joints
.
The
prevalence
of
exertional
leg
pain
among
the
170
FMF
patients
included
in
the
study
was
58
.
2
%
.
Patients
with
exertional
leg
pain
had
significantly
more
joint
attacks
(
74
.
7
%
versus
40
.
8
%
;
P
<
0
.
0001
)
,
fever
attacks
(
35
.
4
%
versus
15
.
5
%
;
P
=
0
.
004
)
,
and
pleuritis
attacks
(
48
.
5
%
versus
29
.
6
%
;
P
=
0
.
013
)
as
well
as
more
attacks
per
year
.
Elevations
of
inflammation
markers
were
significantly
more
frequent
among
the
study
group
(
for
the
erythrocyte
sedimentation
rate
,
44
.
4
%
of
patients
versus
21
.
1
%
of
patients
;
P
=
0
.
016
)
(
for
the
C-
reactive
protein
level
,
48
.
4
%
of
patients
versus
31
.
8
%
of
patients
;
P
=
0
.
013
)
,
and
M
694
V
homozygosity
was
more
prevalent
among
the
study
group
(
45
.
5
%
versus
21
.
1
%
;
P
=
0
.
001
)
.
Signs
compatible
with
enthesopathy
on
MRI
were
observed
in
73
.
5
%
of
patients
in
the
study
group
and
in
33
.
3
%
of
patients
in
the
control
group
(
P
=
0
.
046
)
.
Definite
SpA
was
diagnosed
in
41
.
2
%
of
the
patients
in
the
study
group
compared
to
none
of
the
controls
(
P
=
0
.
07
)
(
odds
ratio
1
.
7
[
95
%
confidence
interval
1
.
2
-
2
.
3
]
)
.
Exertional
leg
pain
is
a
common
manifestation
of
FMF
and
is
a
marker
of
a
more
severe
disease
phenotype
.
Additionally
,
exertional
leg
pain
is
frequently
associated
with
sacroiliitis
and
an
underlying
ankle
enthesopathy
and
should
therefore
be
considered
a
new
feature
of
SpA
.
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
