title: Treatment of primary Sjögren syndrome: a systematic review.
abstract: BACKGROUND
  A variety of topical and systemic drugs are available to treat primary Sjögren syndrome, although no evidence-based therapeutic guidelines are currently available.
  
  
  OBJECTIVE
  To summarize evidence on primary Sjögren syndrome drug therapy from randomized controlled trials.
  
  
  METHODS
  We searched MEDLINE and EMBASE for articles on drug therapy for primary Sjögren syndrome published between January 1, 1986, and April 30, 2010.
  
  
  METHODS
  Controlled trials of topical and systemic drugs including adult patients with primary Sjögren syndrome were selected as the primary information source.
  
  
  RESULTS
  The search strategy yielded 37 trials. A placebo-controlled trial found significant improvement in the Schirmer and corneal staining scores, blurred vision, and artificial tear use in patients treated with topical ocular 0.05% cyclosporine. Three placebo-controlled trials found that pilocarpine was associated with improvements in dry mouth (61%-70% vs 24%-31% in the placebo group) and dry eye (42%-53% vs 26%). Two placebo-controlled trials found that cevimeline was associated with improvement in dry mouth (66%-76% vs 35%-37% in the placebo group) and dry eye (39%-72% vs 24%-30%). Small trials (<20 patients) found no significant improvement in sicca outcomes for oral prednisone or hydroxychloroquine and limited benefits for immunosuppressive agents (azathioprine and cyclosporine). A large trial found limited benefits for oral interferon alfa-2a. Two placebo-controlled trials of infliximab and etanercept did not achieve the primary outcome (a composite visual analog scale measuring joint pain, fatigue, and dryness); neither did 2 small trials (<30 patients) testing rituximab, although significant results were observed in some secondary outcomes and improvement compared with baseline.
  
  
  CONCLUSIONS
  In primary Sjögren syndrome, evidence from controlled trials suggests benefits for pilocarpine and cevimeline for sicca features and topical cyclosporine for moderate or severe dry eye. Anti-tumor necrosis factor agents have not shown clinical efficacy, and larger controlled trials are needed to establish the efficacy of rituximab.
authors.affiliation: Sjögren Syndrome Research Group (AGAUR), Josep Font Laboratory of Autoimmune Diseases, IDIBAPS, Department of Autoimmune Diseases, Hospital Clínic, C/Villarroel, 170, 08036 Barcelona, Spain. mramos@clinic.ub.es
authors.forename: Manuel
authors.initials: M
authors.lastname: Ramos-Casals
authors.forename: Athanasios G
authors.initials: AG
authors.lastname: Tzioufas
authors.forename: John H
authors.initials: JH
authors.lastname: Stone
authors.forename: Antoni
authors.initials: A
authors.lastname: Sisó
authors.forename: Xavier
authors.initials: X
authors.lastname: Bosch
mesh_terms: D006801:Humans; D016032:Randomized Controlled Trials as Topic; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy*