It has been suggested that defects in the modulation of programmed cell death/apoptosis might lead to autoimmune disease, such as Sjögren's syndrome (SS) and systemic lupus erythematosus. In this review some basic information on apoptosis is introduced together with three aspects on apoptosis in relation to SS: i) defective apoptosis could lead to lymphoid cell accumulation and chronic inflammation in exocrine glands; ii) increased apoptosis of epithelial cells might explain the loss of secreting epithelium; and iii) orderly destruction of cellular components might induce autoantibody production. Altogether, the idea that defects in the apoptotic process could be of importance for explaining autoimmune diseases, makes research on the different factors in this pathway valuable for achieving a better understanding of the etiopathogenesis of SS.