Lupus nephritis in childhood.

The manifestations of lupus nephritis (LN) range from minor abnormalities detected on urinalysis to severe renal insufficiency requiring renal replacement therapy. In children, LN is often more severe than in adults. The female to male predominance is not as marked as in adults. The risk of progression to end-stage renal disease in children is 18 to 50%. The majority of children with LN have proteinuria, while the nephrotic syndrome is seen in approximately 50% of affected patients. Children with LN have higher frequency of hypertension which is considered as the most important prognostic clinical finding. The current practice of estimation of complement components, C 3 and C 4 does not adequately reflect disease activity. There are racial differences in renal survival and response to treatment. Arab patients with LN do not exhibit a distinctive serological profile. Lupus nephritis is classified into six groups depending on the severity of the histological lesion. Transformation between the histological classes occurs frequently. Histological outcome predictions have been significantly enhanced by the addition of activity and chronicity indices. Treatment of the LN may be guided by the severity of the renal biopsy appearances. Controversy persists as to the most effective cytotoxic treatment in LN and oral or intravenous (i.v.) cyclophosphamide, azathioprine, cyclosporin, i.v. immunoglobulin, plasma exchange and recently mycophenolate mofetil have been used in different units. Today, children with LN much less commonly go into renal failure. Outcome after renal transplantation of children with end-stage renal disease caused by LN is similar to non-lupus patients. Morbidity of the disease and the treatment remain a major problem.