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Clinical Review
Clinician's Corner
July 28, 2010

Treatment of Primary Sjögren Syndrome: A Systematic Review

Author Affiliations

Author Affiliations: Sjögren Syndrome Research Group (AGAUR), Josep Font Laboratory of Autoimmune Diseases, IDIBAPS, Department of Autoimmune Diseases (Dr Ramos-Casals), and Department of Internal Medicine, Instituto Clínico de Medicina y Dermatologia (Dr Bosch), Hospital Clínic, Barcelona, Spain; Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece (Dr Tzioufas); Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston (Dr Stone); and Primary Care Research Group, IDIBAPS, Primary Care Center Les Corts, GESCLINIC, Barcelona (Dr Sisó).

JAMA. 2010;304(4):452-460. doi:10.1001/jama.2010.1014

Context 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.

Data Sources We searched MEDLINE and EMBASE for articles on drug therapy for primary Sjögren syndrome published between January 1, 1986, and April 30, 2010.

Study Selection 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.