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Anti-SSA/SSB-negative Sjögren's syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution.
Autoimmun Rev. 2015 Nov; 14(11):1019-22.AR

Abstract

OBJECTIVE

This study aims to compare clinical and laboratory features of patients who, while satisfying the American European Consensus Group (AECG) criteria for primary Sjögren's syndrome (SS), do not present the positivity for anti-Ro/SSA and/or anti-La/SSB, with patients that meet the AECG criteria and are positive for anti-Ro/SSA and/or anti-La/SSB.

METHODS

548 patients were selected based on the following criteria, and exclusion of patients negative for histopathology but positive for anti-Ro/SSA and anti-La/SSB: 1. Fulfilment of the AECG criteria, 2. Performance of minor salivary gland biopsy, 3. Search for anti-Ro/SSA and anti-La/SSB, 4. Absence of hepatitis C virus infection. Univariate and multivariate analyses were performed.

RESULTS

Two groups were compared: 342 patients were positive for both the histopathology and for anti-Ro/SSA and/or anti-La/SSB (H-only) and 206 patients were positive for histopathology, but negative for autoantibodies (H+SSA/SSB). The following variables were statistically found to be associated with H+SSA/SSB: younger age at diagnosis (p<0.0001), glandular swelling (p=0.01), purpura (p=0.04), leucopoenia (p=0.0001), lymphoma (p=0.002), low C3 (p=0.04), low C4 (p=0.01), hypergammaglobulinemia (p<0.0001), ANA (p<0.0001), rheumatoid factor (p<0.0001), and serum cryoglobulins (p=0.039). ANA positivity (OR 6.9), hypergammaglobulinemia (OR 5.1), positive rheumatoid factor (OR 2.3), and age at diagnosis (OR 0.97) were also selected by multivariate analyses as associated with H+SSA/SSB.

CONCLUSION

Primary SS negative for anti-Ro/SSA and anti-La/SSB antibodies appears to be characterized by a lower risk of lymphoma and by a lower level of B-cell expansion.

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Authors+Show Affiliations

Quartuccio L
Rheumatology Clinic, Department of Medical and Biological Sciences, Azienda Ospedaliero-Universitaria "S. Maria della Misericordia", Udine, Italy.
Baldini C
Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.
Bartoloni E
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Perugia, Perugia, Italy.
Priori R
Rheumatology Unit, Sapienza University of Rome, Rome, Italy.
Carubbi F
Rheumatology Unit, University of L'Aquila, L'Aquila, Italy.
Corazza L
Rheumatology Clinic, Department of Medical and Biological Sciences, Azienda Ospedaliero-Universitaria "S. Maria della Misericordia", Udine, Italy.
Alunno A
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Perugia, Perugia, Italy.
Colafrancesco S
Rheumatology Unit, Sapienza University of Rome, Rome, Italy.
Luciano N
Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.
Giacomelli R
Rheumatology Unit, University of L'Aquila, L'Aquila, Italy.
Gerli R
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Perugia, Perugia, Italy.
Valesini G
Rheumatology Unit, Sapienza University of Rome, Rome, Italy.
Bombardieri S
Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.
De Vita S
Rheumatology Clinic, Department of Medical and Biological Sciences, Azienda Ospedaliero-Universitaria "S. Maria della Misericordia", Udine, Italy. Electronic address: devita.salvatore@aoud.sanita.fvg.it.

MeSH

BiopsyHumansLymphomaLymphoproliferative DisordersPrevalenceRisk FactorsSjogren's Syndrome

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

26162302

Clinical Trial Links

Biomarkers in Primary Sjögren's Syndrome

Citation

Quartuccio, Luca, et al. "Anti-SSA/SSB-negative Sjögren's Syndrome Shows a Lower Prevalence of Lymphoproliferative Manifestations, and a Lower Risk of Lymphoma Evolution." Autoimmunity Reviews, vol. 14, no. 11, 2015, pp. 1019-22.
Quartuccio L, Baldini C, Bartoloni E, et al. Anti-SSA/SSB-negative Sjögren's syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution. Autoimmun Rev. 2015;14(11):1019-22.
Quartuccio, L., Baldini, C., Bartoloni, E., Priori, R., Carubbi, F., Corazza, L., Alunno, A., Colafrancesco, S., Luciano, N., Giacomelli, R., Gerli, R., Valesini, G., Bombardieri, S., & De Vita, S. (2015). Anti-SSA/SSB-negative Sjögren's syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution. Autoimmunity Reviews, 14(11), 1019-22. https://doi.org/10.1016/j.autrev.2015.07.002
Quartuccio L, et al. Anti-SSA/SSB-negative Sjögren's Syndrome Shows a Lower Prevalence of Lymphoproliferative Manifestations, and a Lower Risk of Lymphoma Evolution. Autoimmun Rev. 2015;14(11):1019-22. PubMed PMID: 26162302.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Anti-SSA/SSB-negative Sjögren's syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution. AU - Quartuccio,Luca, AU - Baldini,Chiara, AU - Bartoloni,Elena, AU - Priori,Roberta, AU - Carubbi,Francesco, AU - Corazza,Laura, AU - Alunno,Alessia, AU - Colafrancesco,Serena, AU - Luciano,Nicoletta, AU - Giacomelli,Roberto, AU - Gerli,Roberto, AU - Valesini,Guido, AU - Bombardieri,Stefano, AU - De Vita,Salvatore, Y1 - 2015/07/08/ PY - 2015/07/01/received PY - 2015/07/06/accepted PY - 2015/7/12/entrez PY - 2015/7/15/pubmed PY - 2016/2/13/medline KW - B cell KW - Lymphoma KW - Rheumatoid factor KW - Sjögren's syndrome SP - 1019 EP - 22 JF - Autoimmunity reviews JO - Autoimmun Rev VL - 14 IS - 11 N2 - OBJECTIVE: This study aims to compare clinical and laboratory features of patients who, while satisfying the American European Consensus Group (AECG) criteria for primary Sjögren's syndrome (SS), do not present the positivity for anti-Ro/SSA and/or anti-La/SSB, with patients that meet the AECG criteria and are positive for anti-Ro/SSA and/or anti-La/SSB. METHODS: 548 patients were selected based on the following criteria, and exclusion of patients negative for histopathology but positive for anti-Ro/SSA and anti-La/SSB: 1. Fulfilment of the AECG criteria, 2. Performance of minor salivary gland biopsy, 3. Search for anti-Ro/SSA and anti-La/SSB, 4. Absence of hepatitis C virus infection. Univariate and multivariate analyses were performed. RESULTS: Two groups were compared: 342 patients were positive for both the histopathology and for anti-Ro/SSA and/or anti-La/SSB (H-only) and 206 patients were positive for histopathology, but negative for autoantibodies (H+SSA/SSB). The following variables were statistically found to be associated with H+SSA/SSB: younger age at diagnosis (p<0.0001), glandular swelling (p=0.01), purpura (p=0.04), leucopoenia (p=0.0001), lymphoma (p=0.002), low C3 (p=0.04), low C4 (p=0.01), hypergammaglobulinemia (p<0.0001), ANA (p<0.0001), rheumatoid factor (p<0.0001), and serum cryoglobulins (p=0.039). ANA positivity (OR 6.9), hypergammaglobulinemia (OR 5.1), positive rheumatoid factor (OR 2.3), and age at diagnosis (OR 0.97) were also selected by multivariate analyses as associated with H+SSA/SSB. CONCLUSION: Primary SS negative for anti-Ro/SSA and anti-La/SSB antibodies appears to be characterized by a lower risk of lymphoma and by a lower level of B-cell expansion. SN - 1873-0183 UR - https://www.unboundmedicine.com/medline/citation/26162302/Anti_SSA/SSB_negative_Sjögren's_syndrome_shows_a_lower_prevalence_of_lymphoproliferative_manifestations_and_a_lower_risk_of_lymphoma_evolution_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1568-9972(15)00151-2 DB - PRIME DP - Unbound Medicine ER -