Active immunological profile is associated with systemic Sjögren's syndrome

J Clin Immunol. 2011 Oct;31(5):840-7. doi: 10.1007/s10875-011-9553-3. Epub 2011 Jul 9.

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.

Conclusion: The subset of patients with active immunological profile is characterized by systemic complications leading to immunosuppressive drug utilization and polyclonal B-cell activation profile.

MeSH terms

  • Adult
  • Antibodies, Antinuclear / blood*
  • Cohort Studies
  • Disease Progression
  • Female
  • Follow-Up Studies
  • France
  • Humans
  • Hypergammaglobulinemia
  • Immunosuppression
  • Lymphoma
  • Male
  • Middle Aged
  • Multiple Organ Failure
  • Prognosis
  • Retrospective Studies
  • Rheumatoid Factor / blood*
  • Sjogren's Syndrome / diagnosis*
  • Sjogren's Syndrome / immunology*
  • Sjogren's Syndrome / mortality
  • Sjogren's Syndrome / physiopathology
  • Sjogren's Syndrome / therapy
  • Survival Analysis

Substances

  • Antibodies, Antinuclear
  • SS-A antibodies
  • SS-B antibodies
  • Rheumatoid Factor