To systematically review the prevalence and risk factors for interstitial lung disease (ILD) in patients with primary Sjögren's syndrome (pSS).
We searched PubMed, Embase, and Cochrane Library from inception to December 2019. Two investigators reviewed records according to predefined criteria. We calculated the pooled prevalence, weighted mean differences (WMDs), odds ratio (OR) and 95% confidence intervals (CIs) using a random effects model, and performed subgroup analysis, sensitivity analysis, and Egger's test.
In 23 studies with 6157 pSS patients, the pooled prevalence of ILD in pSS patients was 13% (95% CI: 9-19). The pSS-ILD prevalence was higher in Asia (20%) than that in Europe (10%). Male gender (OR = 1.92, 95% CI: 1.26-2.95), elder age (WMD = 9.25 years, 95% CI: 2.78-15.72) and higher C-reactive protein (CRP) (WMD = 3.92 mg/L, 95% CI: 0.27-1.61) was associated with ILD in pSS patients.
Interstitial lung disease was prevalent in pSS patients. Elder age, male gender and higher CRP were risk factors for pSS-ILD. Our data highlighted the importance of screening for ILD in high-risk pSS patients.