Lymphoproliferation in autoimmunity and Sjögren's syndrome

Sjögren's syndrome is a chronic inflammatory process involving primarily the exocrine glands. Its association with lymphoma is well documented. A low-grade marginal-zone lymphoma related to mucosa-associated lymphoid tissue is the most common lymphoid neoplasia in Sjögren's syndrome. Among all autoimmune diseases, Sjögren's syndrome is the best tool to clarify the multiple components of autoimmunity and lymphomatogenesis. Herewith, the authors review the literature and discuss the molecular, clinical, histopathologic, and therapeutic aspects of these tumors in Sjögren's syndrome.