SJÖGREN'S SYNDROME


























  • Reference [3]
ACR 2012 Proposed Criteria for Diagnosing SS
  • SS diagnosis is established if patients with symptoms consistent with SS (dry mouth and dry eyes) meet 2 of the following 3 criteria
Criteria 1: Positive serology
  • Positive serology defined as the presence of at least one of the following:
    • Positive serum anti-SSA/Ro
    • Positive serum anti-SSB/La
    • Combination of positive rheumatoid factor and ANA titer ≥ 1:320
Criteria 2: Positive salivary gland biopsy
  • Labial salivary gland biopsy exhibiting focal lymphocytic sialadenitis with a focus score ≥ 1 focus/4mm2
Criteria 3: Positive keratoconjunctivitis sicca (dry eyes) test
  • Keratoconjunctivitis sicca with ocular staining score ≥ 3
  • Assuming that individual is not currently using daily eye drops for glaucoma and has not had corneal surgery or cosmetic eyelid surgery in the last 5 years








JOQUER Trial - Hydroxychloroquine vs Placebo for Primary SS, JAMA (2014) [PubMed abstract]
  • The JOQUER trial enrolled 120 patients with primary SS
Main inclusion criteria
  • Meet the American-European Consensus Group Criteria for primary SS
  • Receiving stable doses of NSAIDs, corticosteroids, pilocarpine, or topical cyclosporine for at least 4 weeks before enrollment
Main exclusion criteria
  • Received rituximab or cyclophosphamide during the 6 previous months or any other immunosuppressant during the previous month
  • Recent serious systemic manifestations (e.g. lymphoma, CNS disease, liver disease, kidney disease)
Baseline characteristics
  • Average age 56 years
  • Female - 91%
  • Median time with symptoms - 5 years
  • Anti-SSA antibodies - 55%
  • Previous systemic involvement - 43%
Randomized treatment groups
  • Group 1 (56 patients) - Hydroxychloroquine (HCQ) 400 mg once daily
  • Group 2 (64 patients) - Placebo
  • Concomitant treatment with a stable dose of nonsteroidal anti-inflammatory drugs, oral corticosteroids, pilocarpine, tear substitutes, and topical cyclosporine were allowed
Primary outcome: Proportion of patients with a 30% or greater reduction between weeks 0 and 24 in scores on 2 of 3 numeric analog scales (from 0 [best] to 10 [worst]) evaluating dryness, pain, and fatigue
Results

Duration: 24 weeks
Outcome HCQ Placebo Comparisons
Primary outcome 17.9% 17.2% OR 1.01, 95% CI [0.37 - 2.78], p=0.98
  • Subgroup analysis showed no efficacy of hydroxychloroquine in patients with anti-SSA autoantibodies, high IgG levels, or systemic involvement

Findings: Among patients with primary Sjögren syndrome, the use of hydroxychloroquine compared with placebo did not improve symptoms during 24 weeks of treatment. Further studies are needed to evaluate longer-term outcomes.