SYSTEMIC AUTOIMMUNE-DISEASE AS A CONSEQUENCE OF DEFECTIVE LYMPHOCYTE DEATH

A major group of systemic autoimmune diseases is associated with abnor mal lymphoproliferation, as a result of defects in the termination of lymphocyte activation and growth. Recent progress has been made in und erstanding the causes and consequences of these abnormalities. At the molecular level, the defects in CD95 and its ligand are only the most obvious reasons for the breakdown of 'clonal contraction' which in fac t requires the participation of multiple gene products, including the IL-2-IL-2-receptor system, to set up a functional apoptotic machinery.