Salivary Gland Pathology in Sjögren's Syndrome

Primary Sjögren syndrome (pSS) can be considered a systemic autoimmune disease with a strong organ bias. The involvement of the exocrine glands is prevalent and drives the pathognomonic manifestations of dryness that define the sicca syndrome. The salivary glands also represent the hub of pSS pathology. Elements belonging to both innate and acquired immune responses have been described at this site that contribute to disease establishment and progression. The interaction between those elements and their relative contributions to the clinical manifestations and lymphoma progression largely remain to be addressed.