Interstitial Lung Disease in Sjogren's Syndrome

Sjogren's syndrome (SS), whether primary, or secondary to other autoimmune rheumatic diseases, is classically associated with the sicca complex of keratoconjunctivits and xerostomia. However, extraglandular manifestations are also relatively common, affecting up to 25% of patients. In this article, we discuss respiratory system involvement in SS, with a focus on interstitial lung disease (ILD). We review the histopathologic variants, clinical features, diagnosis and treatment of SS-ILD. Novel and emerging diagnostic and prognostic biomarkers for SS-ILD are also discussed. We offer an approach to the management of SS-ILD including possible goals of therapy. We conclude by highlighting areas for future research.