Purpose of review: The purpose of this review is to give an update on the understanding of the immune responses involved in the pathogenesis of primary Sjögren's syndrome (pSS), and to highlight recent findings on the underlying molecular and cellular mechanisms at play.
Recent findings: In recent years, genetic studies have confirmed the importance of aberrant type I interferon (IFN) and B cell responses in pSS and highlighted critical pathways involved in disease pathogenesis. In particular, the formation of ectopic lymphoid structures has emerged as an important factor in the establishment of chronic autoimmune responses in target organs. Interestingly, recent studies on viral infection in the context of pSS, as well as findings on the contribution of salivary gland epithelial cells in local immune responses, offer further clues to understand pSS etiology and its target organ specificity. Finally, new evidence brings T cells and natural killer cells under renewed attention as possible important contributors to pSS pathogenesis.
Summary: Progress made during the last few years on the pathogenesis of pSS has been mirrored by clinical trials directed at inhibiting cytokines, B, or T cell responses. Future efforts should focus on identifying additional pSS specific targets and developing methods to help choose optimal therapeutic strategies for the individual patient.