Abstract
Primary Sjögren's syndrome is a systemic autoimmune disease that mainly affects exocrine glands and confers an increased risk of developing lymphomas compared with the general population and even with other autoimmune diseases. It is the most severe comorbidity that occurs in this entity, which increases its mortality. Clinical, serological and histological characteristics have been studied to predict the development of these lymphoproliferative disorders. The most common symptom in this group is unilateral parotid enlargement and they are mainly non-Hodgkin's lymphomas, extranodal marginal lymphoma, derived from mucosa-associated lymphoid tissue or MALT type being the most common between them. It is believed that its development is due to antigenic stimulation of reactive and polyclonal B cells which causes expansion and transition to monoclonal B cells, and together with activation of signaling pathways perpetuates cell proliferation; currently, target therapy directed against the activation of these cells is being studied. Most of these lymphomas have an indolent course and are localized, so they just need surveillance, in case of aggressive or disseminated disease the use of polychemotherapy plus rituximab is preferred.