title: Relation of systemic autoantibodies to the number of extraglandular manifestations in primary Sjögren's Syndrome: a retrospective analysis of 65 patients in the Netherlands.
abstract: OBJECTIVE
  Extraglandular manifestations (EGM) are often seen in patients with primary Sjögren's syndrome and are probably due to a (more) disturbed immune system. Their relation to systemic autoantibodies remains controversial. We hypothesized that positive serology as reflected by the presence of 1 of more systemic autoantibodies is related to the number of EGM.
  
  
  METHODS
  To this purpose, all patients, visiting a large nonacademic teaching hospital, with primary Sjögren's syndrome, according to the revised American-European classification criteria of 2002, were retrospectively analyzed.
  
  
  RESULTS
  In this group of 65 patients, systemic autoantibodies were abundant: anti-Sjögren syndrome A antigen (SSA) and/or anti-Sjögren syndrome B antigen (SSB) (80%), immunoglobulinM-Rheumatoid factor (IgM-RF) (68%), and anti-nuclear antibodies (ANA) (77%). Their presence was often found together and correlated to the presence of hypergammaglobulinemia. There was a statistically significant correlation between the number of systemic autoantibodies and the total number of EGM (P = 0.025). Anti-SSA was the strongest predictor of the presence of EGM (OR = 4.67, P = 0.024).
  
  
  CONCLUSIONS
  These findings indicate that a more disturbed immune system, as reflected by B-cell hyperactivity, with autoantibody formation and hypergammaglobulinemia, is associated with more systemic manifestations in patients with primary Sjögren's syndrome.
authors.affiliation: Department of Rheumatology, St. Antonius Hospital, Nieuwegein, The Netherlands. borg@antoniusziekenhuis.nl
authors.forename: Evert-Jan
authors.initials: EJ
authors.lastname: ter Borg
authors.forename: Anna P
authors.initials: AP
authors.lastname: Risselada
authors.forename: Johannes C
authors.initials: JC
authors.lastname: Kelder
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D008297:Male; D008875:Middle Aged; D009426:Netherlands; D012189:Retrospective Studies; D012217:Rheumatoid Factor / Q000097:blood; D012469:Salivary Glands / Q000276:immunology* / Q000503:physiopathology; D012859:Sjogren's Syndrome / Q000097:blood / Q000276:immunology* / Q000503:physiopathology
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D007075:Immunoglobulin M; C035356:SS-A antibodies; C035357:SS-B antibodies; D012217:Rheumatoid Factor