Lupus nephritis in children

Abstract

Summary: Relatively few studies of children with lupus nephritis have been published. the prognosis of children with lupus nephritis is ominous for those with diffuse proliferative glomerulonephritis and active interstitial inflammation. to evaluate the clinical course, histopathology and prognosis of lupus nephritis in children, to identify the risk factors for renal failure and mortality, and to share our experience in managing lupus nephritis in children, 167 children under 18 years of age with lupus nephritis at Veterans General Hospital‐Taipei, Taiwan from 1979 to 1991 were retrospectively studied. All patients received a renal biopsy and follow‐up biopsies were performed on 36 children. the clinical and serologic parameters at the time of renal biopsy were recorded. There were 55 (33%) patients with class II, 30 (18%) with class III, 69 (41.3%) with class IV and 13 (7.8%) with class V nephritis based on initial biopsy. the mean follow‐up time was 59 months. Follow‐up biopsies were histologically stationary in 29 patients, progressive in five and improved in two. the results revealed that those with persistent hypertension, anaemia, increased serum creatinine concentration, and decreased creatinine clearance at initial biopsy were more prone to develop renal failure. Low titre of CH50 haemolytic assay appeared to be a poor prognostic indicator. the overall renal and patient 5 year survival rates were 93.1% (135/145) and 91.08% (143/157), respectively. They were 87.7% (50/57) and 82% (55/67), respectively, in patients with class IV proliferative glomerulonephritis. the prognosis of children with class IV nephritis in this study was better than that reported previously. All children surviving without renal failure were maintaining their normal lives with little organ dysfunction. the improved results may be due to earlier renal biopsy for precise histopathologic definition, better supportive care, and selective use of aggressive therapy, including methylprednisolone pulse therapy, intravenous cyclophosphamide, intravenous prostaglandin E1 therapy, high‐dose intravenous gammaglobulin therapy, and cyclosporine A for those with high risk factors.