Abstract
Deficiency in the classical pathway complement components displays a hierarchical association with the development of systemic lupus erythematosus (SLE). In addition, SLE causes consumption of complement. C1q- and C4-deficient mice develop a lupus-like disease and exhibit impaired clearance of apoptotic cells. The autoantigens targeted in SLE have been localised to the surface of apoptotic cells, which may be the source of these antigens. Although apoptosis was originally thought to be an immunologically inert process, dendritic cells can present epitopes derived from apoptotic cells, and immunization with apoptotic cells leads to the generation of autoantibodies. These findings taken together indicate that a defect in complement-dependent clearance of apoptotic cells may increase susceptibility to the development of autoimmunity.