In contrast, some children present with severe life threatening disease manifestations. SLE is an unpredictable disease and may be fatal with or without renal involvement. Renal involvement is the most prevalent life threatening complication of SLE . For most children with significant renal involvement this takes the form of hematuria or proteinuria . There are many extra-renal complications of childhood SLE .
Neurologic involvement is the most difficult.
When a child or adolescent with SLE becomes noncompliant and difficult, the family and physicians often ascribe the behavior to the psychological problems associated with adolescence and chronic illness or chronic corticosteroid therapy. Some times it is caused by the lupus itself. Although SLE remains an infrequent disease in general pediatric practice, mild cases are more frequent than previously recognized. Our understanding of the role of genetics and environmental agents in the pathogenesis of SLE has improved over the past ten years. In addition, the past ten years have seen refinements in the use of immunosuppressive regimens such as cyclophosphamide which have lead to both improved quality of life and improved survival for children with active SLE unresponsive to corticosteroids.