Prevalence of Interstitial Lung Disease in Patients with Primary Sjogren’s Syndrome: A Systematic Review and Meta-analysis of Observational Studies

Abstract

Background: Interstitial Lung Disease (ILD) is the most common pulmonary manifestation in patients with primary Sjögren’s syndrome. Therefore, this study aimed to investigate the prevalence of ILD in patients with primary Sjögren’s syndrome (pSS). Materials and Methods: This study searched databases ProQuest, PubMed, Web of Science, Cochrane, Embase, and the search engine Google Scholar until February 8, 2025. Studies were combined using the sample size and variance of each study. The heterogeneity of the studies was examined using the Q Cochrane test and the I2 index. . Results: 34 observational studies, with a total of 9535 individuals, showed that the prevalence of ILD in all patients with pSS was 23.5% (95%CI: 18.6%, 28.4%), in women 31.2% (95%CI: 18.4%, 44.1%), and in men 45.5% (95%CI: 23.6%, 67.4%). Moreover, the prevalence of ILD in patients with pSS in the age group of 30 to 39 years was 22.2% (95%CI: 18.9%, 26%), 40 to 49 years 10.8% (95%CI: 8.7%, 12.9%), 50 to 59 years 27.6% (95%CI: 17.9%, 37.4%), and 60 to 69 years 23.3% (95%CI: 17.9%, 28.7%). Also, the prevalence of ILD in patients with pSS in case-control studies was 15.6% (95%CI: 7.1%, 24.1%), in cohort studies 25.7% (95%CI: 19%, 32.4%), and in cross-sectional studies 21.4% (95%CI: 11.9%, 31%). However, the prevalence of non-specific interstitial pneumonia (NSIP) was 48.8% (95%CI: 41.4%, 56.2%), usual interstitial pneumonia (UIP) 15% (95%CI: 10.5%, 19.5%), LIP 10.8% (95%CI: 6.6%, 15%), and organizing pneumonia (OP) 9.4% (95%CI: 5.4%, 13.4%). Conclusion: One out of every four patients with pSS has ILD, and the prevalence of ILD was higher in men than in women. Moreover, the most common type of ILD was NSIP, followed by UIP, OP, and LIP, and the highest prevalence of ILD was in the age group of 50 to 59 years.