title: Active immunological profile is associated with systemic Sjögren's syndrome.
abstract: BACKGROUND
  The aim of this paper was to study the evolution of primary Sjögren's syndrome (pSS) immunological profile, its impact on pSS activity and long-term evolution in a bicentric cohort of French patients with pSS (n = 445, mean age 53.6 ± 14 years, mean follow-up 76.1 ± 51 months).
  
  
  METHODS
  This is a retrospective cohort study.
  
  
  RESULTS
  Two hundred twelve patients were Sjögren's syndrome A (SSA) positive, and 131 were both SSA and Sjögren's syndrome B (SSB) positive. Sixty-eight patients (15%) had cryoglobulinemia. Active systemic profile (i.e., hypergammaglobulinemia, rheumatoid factor (RF), and anti-Sjögren's syndrome A (anti-SSA), anti-Sjögren's syndrome B (anti-SSB) positivity), associated with multisystemic involvement, leads to an increased utilization of corticosteroid and hydroxychloroquine. Multivariate analysis pointed out independent statistical association between hypergammaglobulinemia, anti-SSA, anti-SSB, and RF. Cryoglobulinemia is associated with multi-systemic involvement, lymphoma, and pSS-related death.
  
  
  CONCLUSIONS
  The subset of patients with active immunological profile is characterized by systemic complications leading to immunosuppressive drug utilization and polyclonal B-cell activation profile.
authors: Department of Internal Medicine, Limoges University Hospital, Limoges, France.
authors: Clothilde
authors: C
authors: Martel
authors: Guillaume
authors: G
authors: Gondran
authors: David
authors: D
authors: Launay
authors: Fabrice
authors: F
authors: Lalloué
authors: Sylvain
authors: S
authors: Palat
authors: Marc
authors: M
authors: Lambert
authors: Kim
authors: K
authors: Ly
authors: Veronique
authors: V
authors: Loustaud-Ratti
authors: Holly
authors: H
authors: Bezanahary
authors: Eric
authors: E
authors: Hachulla
authors: Marie Odile
authors: MO
authors: Jauberteau
authors: Elisabeth
authors: E
authors: Vidal
authors: Pierre Yves
authors: PY
authors: Hatron
authors: Anne Laure
authors: AL
authors: Fauchais
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood*; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D006942:Hypergammaglobulinemia; D007165:Immunosuppression Therapy; D008223:Lymphoma; D008297:Male; D008875:Middle Aged; D009102:Multiple Organ Failure; D011379:Prognosis; D012189:Retrospective Studies; D012217:Rheumatoid Factor / Q000097:blood*; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000276:immunology* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D016019:Survival Analysis
chemical_list: D000974:Antibodies, Antinuclear; C035356:SS-A antibodies; C035357:SS-B antibodies; D012217:Rheumatoid Factor