ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2183

Common Features in Lymphoproliferative Complications in the Course of Primary Sjögren’s Syndrome: Results From a Multicenter Cohort of 1170 Patients

Luca Quartuccio1, Chiara Baldini2, Roberta Priori3, Elena Bartoloni Bocci4, Francesco Carubbi5, Miriam Isola6, Marta Maset7, Sara Salvin7, Nicoletta Luciano2, Giovanna Picarelli8, Alessia Alunno9, Roberto Giacomelli10, Roberto Gerli11, Guido Valesini12, Stefano Bombardieri13 and Salvatore De Vita1, 1Rheumatology, DSMB, University Hospital Santa Maria della Misericordia, Udine, Italy, 2University of Pisa, Rheumatology Unit, Pisa, Italy, 3Department of Internal Medicine and Medical Specialties, Sapienza University, Rome, Italy, 4Rheumatology Unit, Department of Clinical & Experimental Medicine, University of Perugia, Perugia, Italy, 5Rheumatology Clinic, University of L'Aquila, L'Aquila, Italy, 6Institute of Statistics, University of Udine, Udine, Italy, 7Rheumatology Clinic, DSMB, University of Udine, Italy, Udine, Italy, 8Rheumatology Unit, Sapienza University of Rome, Rome, Italy, 9Clinical and Experimental Medicine, Rheumatology Unit, University of Perugia, Perugia, Italy, 10Rheumatology Unit, University of Aquila, L'Aquila, Italy, 11Rheumatology Unit, University of Perugia, Perugia, Italy, 12Clinica e Terapia Medica, Sapienza, Universita di Roma, Rome, Italy, 13Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: , ,

Session Information

Title: Sjögren's Syndrome - Clinical

Session Type: Abstract Submissions (ACR)

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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