Current Evidence SLE is a multisystem disease that is both pleomorphic in its presentation and variable in its clinical course In many ways, it is the quintessential autoimmune disease, with antibodies to cellular constituents causing immune-mediated attack on various organs, including the skin, joints, peripheral and central nervous system, kidneys, and serosal surfaces In children, the disease is more severe, with a higher incidence of renal and neurologic involvement The classification system for SLE was revised in 2012, reflecting a harmonization between the newer criteria of the Systemic Lupus International Collaborating Clinics (SLICC) group and the criteria of the American College of Rheumatology (ACR), which had been the standard classification system for decades Table 101.1 lists the ACR criteria and the updated revised SLICC criteria and definitions This newer classification differs from the 1997 ACR criteria in two significant ways First, the SLICC criteria were expanded to include 17 individual elements, rather than 11, thus greatly expanding the breadth of the diagnosis Secondly, the diagnosis rests on the presence of one immunologic criterion as well as the presence of at least one clinical criterion, rather than the previous format that relied on only clinical symptoms in some cases The SLICC criteria have improved sensitivity (97%) but decreased specificity (84%) when compared to the 1997 ACR criteria