Systemic lupus erythematosus is a prototype of systemic autoimmune disease which is characterized by polyclonal B cell activation and production of various autoantibodies, especially antibodies against nucleosome. The pathogenesis of lupus does not depend on a single gene defect but depend on multiple genetic and environmental factors. Lately defects of apoptosis have been focused as a potential etiologic factor of systemic autoimmune diasease. Apoptosis is an active programmed cell death which removes unwanted cells without any inflammatory sequelae. Cells of immune system are programmed to be deleted unless recruited into appropriate immune response. The fate of certain cell may be controlled by the balance between cell death signals and survival signals for each cells. The importance of these pathways in maintaining tolerance is highlighted by the development of lupus-like disease in three different mouse strains that have spontaneous mutations in the Fas or its ligand. We have a lot of reports on the relationship between apoptosis and systemic lupus erythematosus in terms of pathogenesis after then. We need to review all information on apoptosis and lupus and find appropriate direction toward research and clinical aspect, even though we don t have complete knowledge on signal transduction and regulators of apoptosis. Here relationships between systemic lupus erythematosus and apoptosis were reviewed on a few different viewpoints : 1) too little apoptosis of immune cell, 2) accelerated spontaneous apoptosis and nucleosome secretion, 3) defective phagocytosis of macrophage, 4) relationship between antiphospholipid antibody and apoptosis.