title: Anti-SSA/SSB-negative Sjögren's syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Rheumatology Clinic, Department of Medical and Biological Sciences, Azienda Ospedaliero-Universitaria "S. Maria della Misericordia", Udine, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Quartuccio
authors.affiliation: Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Baldini
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Perugia, Perugia, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Bartoloni
authors.affiliation: Rheumatology Unit, Sapienza University of Rome, Rome, Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: Priori
authors.affiliation: Rheumatology Unit, University of L'Aquila, L'Aquila, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Carubbi
authors.affiliation: Rheumatology Clinic, Department of Medical and Biological Sciences, Azienda Ospedaliero-Universitaria "S. Maria della Misericordia", Udine, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Corazza
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Perugia, Perugia, Italy.
authors.forename: Alessia
authors.initials: A
authors.lastname: Alunno
authors.affiliation: Rheumatology Unit, Sapienza University of Rome, Rome, Italy.
authors.forename: Serena
authors.initials: S
authors.lastname: Colafrancesco
authors.affiliation: Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Luciano
authors.affiliation: Rheumatology Unit, University of L'Aquila, L'Aquila, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Giacomelli
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Perugia, Perugia, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Gerli
authors.affiliation: Rheumatology Unit, Sapienza University of Rome, Rome, Italy.
authors.forename: Guido
authors.initials: G
authors.lastname: Valesini
authors.affiliation: Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Bombardieri
authors.affiliation: 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.
authors.forename: Salvatore
authors.initials: S
authors.lastname: De Vita
mesh_terms: D001706:Biopsy; D006801:Humans; D008223:Lymphoma / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology; D015995:Prevalence; D012307:Risk Factors; D012859:Sjogren's Syndrome / Q000150:complications / Q000453:epidemiology / Q000276:immunology*
keywords: B cell; Lymphoma; Rheumatoid factor; Sjögren's syndrome