Background There is conflicting evidence regarding prognosis in patients with primary Sjögren's syndrome (pSS).
Objectives We estimated rate, risk factors and causes of mortality in patients with pSS through a systematic review and meta-analysis.
Methods Through a systematic review of multiple databases through October 2014, we identified cohort studies reporting relative risk (compared to standardized population), risk factors and causes of mortality in patients with pSS. We estimated summary risk ratios (RR) with 95% confidence intervals (CI), using random effects model.
Results We identified 10 studies with 7,888 patients (91% females) with pSS, of whom 682 patients died over a median average follow-up of 9 years. Pooled standardized mortality ratio in patients with pSS was 1.38 (95% CI, 0.94-2.01). Leading causes of mortality were cardiovascular diseases, solid-organ and lymphoid malignancies and infections. Risk factors associated with mortality were: advanced age at diagnosis (RR, 1.09; 95% CI, 1.07-1.12), males (RR, 2.18; 95% CI, 1.45-3.27), parotid enlargement (RR, 1.81; 95% CI, 1.02-3.21), abnormal parotid scintigraphy (RR, 2.96; 95% CI, 1.36-6.45), extraglandular involvement (RR, 1.77; 95% CI, 1.06-2.95), vasculitis (RR, 7.27; 95% CI, 2.70-19.57), anti-SSB positivity (RR, 1.45; 95% CI, 1.03-2.04), low C3 (RR, 2.14; 95% CI, 1.38-3.32) and C4 (RR, 3.08; 95% CI, 2.14-4.42) and cryoglobulinemia (RR, 2.62; 95% CI, 1.77-3.90).
Conclusions pSS is not associated with an increase in all-cause mortality, as compared to the general population. However, a subset of patients with extraglandular involvement, vasculitis, hypocomplementemia and cryoglobulinemia may be at increased risk of mortality, and require close follow-up.
Disclosure of Interest None declared
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