159 Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a multisystem, inflammatory, autoimmune disease that can affect any organ system and is characterized by the presence of circulating antinuclear antibodies, especially antibodies to native (double-stranded) DNA. Its clinical manifestations are widely variable, and its course is unpredictable. If left untreated, SLE is often progressive and has a significant fatality rate. It is estimated that 15–20% of patients with SLE have their onset before 16 years of age. Although clinical and laboratory features in juvenile SLE are similar to those that are seen in adults, SLE that begins in childhood has been considered more severe than SLE with onset during adulthood. Furthermore, children diagnosed with SLE may need high-dose corticosteroids and immunosuppressive agents for disease control more often than do their adult counterpart. In 1971, the American College of Rheumatology (formerly American Rheumatism Association) developed a set of criteria for the classification of SLE, which were revised in 1982 and modified in 1997 (> Table 159.1). SLE is established when a patient fulfills at least 4 of the 11 criteria. The criteria can be present simultaneously or occur with time along the disease course. The 1982 criteria have been determined to have a sensitivity of 96% and a specificity of 100% in juvenile SLE.