Seronegative Sjögren's Syndrome Is Characterized By a Lower Prevalence of B-Cell Related Clinical Manifestations and a Lower Biologic Systemic Activity

Luca Quartuccio¹, Chiara Baldini^2,3,4, Elena Bartoloni Bocci⁵, Roberta Priori⁶, Francesco Carubbi⁷, Laura Corazza¹, Alessia Alunno⁸, Serena Colafrancesco⁶, Nicoletta Luciano², Roberto Gerli⁹, Roberto Giacomelli¹⁰, Guido Valesini¹¹, Stefano Bombardieri¹² and Salvatore De Vita^13,14, ¹S. Maria della Misericordia, University of Udine, Italy, Udine, Italy, ²Internal Medicine, University of Pisa, Pisa, Italy, ³University of Pisa, Rheumatology Unit, Pisa, Italy, ⁴Department of Internal Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy, ⁵Rheumatology Unit, Department of Medicine, University of Perugia, Pila, Italy, ⁶Rheumatology Unit, Sapienza University of Rome, Rome, Italy, ⁷Rheumatology Clinic, University of L'Aquila, L'Aquila, Italy, ⁸Clinical and Experimental Medicine, Rheumatology Unit, University of Perugia, Perugia, Italy, ⁹Rheumatology Clinic, University of Perugia, Perugia, Italy, ¹⁰University of L'Aquila, L'Aquila, Italy, ¹¹Internal Medicine and Medical Specialties Department, Policlinico Umberto I, La Sapienza University of Rome, Roma, Italy, ¹²Rheumatology Unit, University of Pisa, Italy, Pisa, Italy, ¹³Santa Maria della Misericordia Hospital, University of Udine, Udine, Italy, ¹⁴Rheumatology, DSMB, University Hospital Santa Maria della Misericordia, Udine, Italy

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: autoantibodies, complement and cryoglobulinemia, Rheumatoid Factor, Sjogren's syndrome

Session Information

Date: Sunday, November 8, 2015

Title: Sjögren's Syndrome Poster I: Clinical Insights into Sjögren's Syndrome

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: The American-European Consensus Group (AECG) Criteria (1) for Sjögren’s syndrome (SS) require the presence of at least one of the following two conditions: the histopathology (focus score > / = 1) or the presence of anti- SSA and / or SSB antibodies. The study aims to compare patients who, while satisfying the AECG criteria, do not present the positivity for anti-SSA/SSB antibodies, with patients that meet the AECG criteria and are positive for anti-SSA/SSB antibodies.

Methods: 598 patients were selected from the database of the Italian study group on SS (2) based on the following criteria: 1. Fulfillment of the AECG criteria for SS, 2. Execution of the biopsy of the minor salivary glands, 3. Search for anti-SSA/SSB antibodies, 4. Absence of hepatitis C virus infection. Univariate and multivariate analyses were performed. Differences were considered significant for P value <0.05. Variables with P <0.1, were then selected for the multivariate analysis.

Results: 206 patients were positive for histopathology, but negative for autoantibodies (group 1), 342 were positive for both the histopathology and for anti-SSA/SSB antibodies (group 2), and finally 50 patients were negative for the histopathology and positive for anti-SSA/SSB antibodies (group 3). This group has been excluded from the comparisons for the small size.

We found the following statistically significant differences between the two groups of patients (group 1 vs. group 2): age at diagnosis [mean ± standard error (SE)] (55 ± 0.8 vs. 48 ± 0.8, p <0.0001), glandular swelling (52/206 vs. 121/342, p = 0.013), skin involvement (14/206 vs. 47/342, p = 0.01), hematologic involvement (47/206 vs. 124/342, p = 0.001), leukopenia (36/170 vs. 110/230, p = 0.0001), lymphoma (22/342 vs. 2/206, p = 0.002), low C3 (24/187 vs. 63/316, p = 0.04), low C4 (12/187 vs. 43/316, p = 0.013), hypergammaglobulinemia (37/200 vs. 214/336, p <0.0001), ANA (323 vs. 133/202 / 337, p <0.0001), rheumatoid factor (59/198 vs. 224/331, p <0.0001), serum cryoglobulins (24/278 vs. 5/149, p = 0.039). The duration of follow-up (mean ± SE) was higher in the group 2 (4.0 ± 0.4 vs. 5.6 ± 0.3, p = 0.001).

By contrast, there were no statistically significant differences for the following variables: sex, dry eye, dry mouth, joint involvement, Raynaud’s phenomenon, purpura, kidney, lung, central and peripheral nervous system involvements, stomach, heart or muscle involvements, fibromyalgia, and the presence of serum monoclonal component, or prevalence of thyroiditis.

By multivariate analysis the following variables were selected as associated with the group 2: antinuclear antibodies (OR 6.9 95% CI 3.2 to 15.1), hypergammaglobulinemia (OR 5.1 95% CI 2.9 to 9.0 ), rheumatoid factor (OR 2.3 95% CI 1.4 to 3.9), age at diagnosis (OR 0.9 95% CI from 0.96 to 0.99).

Conclusion: B-cell related clinical manifestations and biologic activity appears less relevant in seronegative SS. Personalized treatment in SS as well as trial design of new therapies for SS should take into account these differences for patients’ recruitment beyond the fulfillment of the classification criteria.

Disclosure: L. Quartuccio, None; C. Baldini, None; E. Bartoloni Bocci, None; R. Priori, None; F. Carubbi, None; L. Corazza, None; A. Alunno, None; S. Colafrancesco, None; N. Luciano, None; R. Gerli, None; R. Giacomelli, None; G. Valesini, None; S. Bombardieri, None; S. De Vita, None.

To cite this abstract in AMA style:

Quartuccio L, Baldini C, Bartoloni Bocci E, Priori R, Carubbi F, Corazza L, Alunno A, Colafrancesco S, Luciano N, Gerli R, Giacomelli R, Valesini G, Bombardieri S, De Vita S. Seronegative Sjögren’s Syndrome Is Characterized By a Lower Prevalence of B-Cell Related Clinical Manifestations and a Lower Biologic Systemic Activity [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/seronegative-sjogrens-syndrome-is-characterized-by-a-lower-prevalence-of-b-cell-related-clinical-manifestations-and-a-lower-biologic-systemic-activity/. Accessed .

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