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Systemic sclerosis and prevalence of monoclonal immunoglobulin.
[waldenström macroglobulinemia]
The
purpose
of
this
study
was
to
estimate
the
prevalence
of
monoclonal
immunoglobulin
(
MIg
)
among
patients
with
systemic
sclerosis
(
SSc
)
according
to
the
capillary
electrophoresis
or
immunofixation
method
of
detection
and
to
search
for
any
related
clinical
correlations
.
Retrospective
multicenter
comparison
of
capillary
electrophoresis
and
immunofixation
results
in
SSc
patients
and
of
the
characteristics
of
patients
with
and
without
MIg
.
The
study
included
244
SSc
patients
(
216
women
and
28
men
,
mean
age
:
55
±
14
years
)
.
Median
time
since
SSc
diagnosis
was
51
months
[
0
-
320
]
;
disease
was
diffuse
in
48
%
of
cases
.
Ten
percent
of
patients
had
cancer
,
including
Waldenström
macroglobulinemia
(
n
=
1
)
and
multiple
myeloma
(
n
=
3
)
.
Capillary
electrophoresis
showed
a
γ-globulin
anomaly
in
41
%
of
cases
,
and
immunofixation
in
18
%
:
MIg
(
13
.
5
%
)
and
restriction
of
heterogeneity
(
4
.
5
%
)
.
Capillary
electrophoresis
failed
to
detect
60
%
of
the
33
MIg
patients
.
Measurable
MIg
concentrations
were
obtained
from
7
patients
.
MIg
patients
were
significantly
older
at
SSc
diagnosis
than
those
without
MIg
(
p
=
0
.
002
)
,
had
a
lower
diffusing
capacity
(
p
=
0
.
002
)
,
a
higher
prevalence
of
pulmonary
hypertension
and
cancer
(
p
=
0
.
002
)
and
were
more
frequently
positive
for
anti-
mitochondrial
and
anti-
beta
2
-
glycoprotein-
I
antibodies
(
p
=
0
.
03
and
p
=
0
.
02
,
respectively
)
.
Multivariate
analyses
showed
that
only
age
at
test
[
hazard
ratio
1
.
03
(
95
%
CI
,
1
.
00
-
1
.
07
,
p
=
0
.
04
)
]
and
presence
of
cancer
[
hazard
ratio
4
.
46
(
95
%
CI
,
1
.
6
-
12
.
4
,
p
=
0
.
004
)
]
were
associated
with
MIg
.
Immunofixation
detected
a
high
prevalence
of
MIg
among
SSc
patients
especially
in
patients
aged
50
-
years
or
older
.
MIg
was
not
detected
by
the
standard
capillary
electrophoresis
in
60
%
of
cases
and
was
significantly
associated
with
cancer
.
Diseases
Validation
Diseases presenting
"pulmonary hypertension"
symptom
acute rheumatic fever
alpha-thalassemia
cohen syndrome
congenital diaphragmatic hernia
heparin-induced thrombocytopenia
homocystinuria without methylmalonic aciduria
lymphangioleiomyomatosis
malignant atrophic papulosis
systemic capillary leak syndrome
waldenström macroglobulinemia
This symptom has already been validated
