Systemic Lupus Erythematosus: Etiology, Pathogenesis, Clinical Manifestations, and Management

The etiology of systemic lupus erythematosus (SLE) remains unknown. Although great strides are being made toward clarifying the immune dysregulation seen in SLE, clinical disease expression is undoubtedly the end result of varied environmental and immunologic stimuli acting on a genetically predisposed individual. Abnormalities of the T cells, B cells, dendritic cells, Fcγ receptors, proinflammatory cytokines, the complement pathway, and apoptosis have all been found to play a role in the pathogenesis of SLE. As with any chronic medical condition, the treatment of SLE in children is a particular challenge. As children enter adolescence and early adulthood, the desire for a seemingly “normal” life with a sense of independence from parents and medical authority figures may dominate, and compliance may wane. This is particularly true for teenage patients for whom body image issues come to the forefront when faced with the effects of corticosteroid therapy. Treatment of SLE must be individually tailored to each patient's clinical manifestations. Corticosteroids remain the first line of treatment for SLE. However, depending upon the extent and severity of internal organ involvement, medication regimens can range from low-dose corticosteroids and antimalarials to inpatient treatment with pulse methylprednisolone and cytotoxic medications such as cyclophosphamide and rituximab.