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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
"cancer"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
alpha-thalassemia
benign recurrent intrahepatic cholestasis
cadasil
canavan disease
carcinoma of the gallbladder
cholangiocarcinoma
coats disease
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
familial hypocalciuric hypercalcemia
familial mediterranean fever
gm1 gangliosidosis
heparin-induced thrombocytopenia
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
hodgkin lymphoma, classical
inclusion body myositis
junctional epidermolysis bullosa
kabuki syndrome
kallmann syndrome
kindler syndrome
lamellar ichthyosis
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
pleomorphic liposarcoma
primary effusion lymphoma
proteus syndrome
pyomyositis
pyruvate dehydrogenase deficiency
severe combined immunodeficiency
sneddon syndrome
systemic capillary leak syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated
