Background/Purpose:
To describe the prevalence of lymphoproliferative complications (defined as B-cell lymphoma or definite conditions predisposing to lymphoma, i.e, cryoglobulinemic vasculitis (CV) and major salivary gland swelling) in the course of primary Sjögren’s syndrome (pSS) in a large cohort of patients followed in five Rheumatologic Centres.
Methods:
Demographic, clinical, laboratory and histopathologic data in 1170 pSS were retrospectively collected according to a standard protocol. Univariate and multivariate analyses were performed.
Results:
Prevalence of lymphoma in this SS cohort was 4.4% (51/1170), prevalence of CV was 3.9% (33/850), and prevalence of salivary gland swelling and/or myoepithelial syaladenitis was 30.9% (362/1170). Salivary gland swelling and/or MESA, CV and lymphoma shared many laboratory features, i.e., positive rheumatoid factor, hypocomplementemia and leucopenia, as well as a the presence of purpura as clinical hallmark of the circulating immune complexes (table 1). Interestingly, polyclonal hypergammaglobulinemia was strictly associated with salivary gland swelling, but it was not associated with CV or lymphoma; on the other hand, serum monoclonal component was significantly associated with CV or lymphoma, but not with salivary gland swelling and/or MESA (table 1). Younger age was associated with salvary gland swelling and/or MESA, while male sex with an increased risk of lymphoma (OR 5.4 95% CI 2.4-11.7) (table 1). By multivariate analyses, glandular swelling (OR 8.9 95%CI 4.0-19.9, p<0.0001), low C4 (OR 6.2 95%CI 3.0-12.8, p<0.0001), anti-La (OR 3.8 95%CI 1.8-7.9, p<0.0001), leukopenia (OR 2.8 95%CI 1.4-5.7) and monoclonal component (OR 3.2 95%CI 1.3-7.9, p=0.01) were strictly related to lymphoma, while peripheral nervous system involvement (OR 6.8 95%CI 2.1-21.6, p=0.001), low C4 (OR 7.3 95%CI 2.7-19.8, p<0.0001) and leukopenia (OR 6.5 95%CI 2.3-17.9) with CV, and, finally, younger age (OR 0.97 95%CI 0.96-0.98, p<0.0001), lymphoma (OR 7.9 95%CI 3.9-16.1, p<0.0001) and hypergammaglobulinemia (OR 1.5 95%CI 1.1-2.0, p=0.007) with salivary gland swelling and/or MESA.
|
Feature |
SS with salivary gland swelling and/or MESA (362/1170: 30.9%)
P value |
SS with cryoglobulinemic vasculitis (33/850: 3.9%)
P value |
SS with lymphoma (51/1170: 4.4%)
P value |
|
Age (yrs) |
<0.0001 (for youngers) |
ns |
ns |
|
Sex |
ns |
ns |
<0.0001 (for males) |
|
Salivary gland swelling and/or MESA |
NA |
ns |
<0.0001 |
|
Cryoglobulinemic vasculitis |
0.009 |
NA |
0.001 |
|
Lymphoma |
<0.0001 |
<0.0001 |
NA |
|
Purpura |
0.03 |
<0.0001 |
0.02 |
|
Peripheral nervous system |
ns |
<0.0001 |
0.04 |
|
Renal involvement |
ns |
0.007 |
ns |
|
Fibromyalgia |
ns |
0.007 |
ns |
|
ANA positivity |
0.001 |
ns |
ns |
|
Anti-SSA positivity |
<0.0001 |
ns |
0.005 |
|
Anti-SSB positivity |
<0.0001 |
ns |
<0.0001 |
|
Rheumatoid factor positivity |
<0.0001 |
<0.0001 |
<0.0001 |
|
Presence of serum cryoglobulins |
ns |
<0.0001 |
<0.0001 |
|
Low C3 |
0.003 |
<0.0001 |
0.02 |
|
Low C4 |
<0.0001 |
<0.0001 |
<0.0001 |
|
Leukopenia (<3000/mmc) |
0.004 |
<0.0001 |
<0.0001 |
|
Hypergammaglobulinemia (>1.7 g/l) |
<0.0001 |
ns |
ns |
|
Presence of serum M-component |
ns |
<0.0001 |
<0.0001 |
Conclusion:
Salivary gland swelling, CV and B-cell lymphoma are consequent to B-cell deregulation in SS. B-cell clonal proliferation from polyclonal to monoclonal likely occurs in the pathologic target tissue of SS (i.e., salivary gland MALT tissue), predisposing to CV and/or to B-cell lymphoma.
Disclosure:
L. Quartuccio,
None;
C. Baldini,
None;
R. Priori,
None;
E. Bartoloni Bocci,
None;
F. Carubbi,
None;
M. Isola,
None;
M. Maset,
None;
S. Salvin,
None;
N. Luciano,
None;
G. Picarelli,
None;
A. Alunno,
None;
R. Giacomelli,
None;
R. Gerli,
None;
G. Valesini,
None;
S. Bombardieri,
None;
S. De Vita,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/common-features-in-lymphoproliferative-complications-in-the-course-of-primary-sjogrens-syndrome-results-from-a-multicenter-cohort-of-1170-patients/