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1812 Outcome of Childhood Systemic Lupus Erythematosus (SLE) with Lupus Nephritis (LN)
  1. M Ilyas1,
  2. A Salahuddin1,
  3. RC Butterfield2,
  4. A Tolaymat1
  1. 1Pediatrics
  2. 2Center for Health Equity and Quality Research, University of Florida, Jacksonville, FL, USA

Abstract

A total of 46 patients with SLE-LN were studied (91% females; mean age 13.0 years; mean follow-up duration 5.9 years). Initial renal biopsy showed that out of 46 patients, 2 (4.3%) had Class I, 14 (30.4%) had Class II, 8 (17.4%) had Class III, 20 (43.3%) had Class IV and 2 (4.3%) had Class V lupus nephritis. Based on the renal histopathology and clinical presentation, 23 (50%) patients received intermittent intravenous cyclophosphamide bolus with prednisone and Azathioprine (AZT) or Mycophenolate Mofetil (MMF). The remaining 23 (50%) patients were treated with corticosteroids alone or in combination with AZT or MMF.

Follow up renal biopsies were performed on 21 patients, 4 patients showed no change in histology, 8 patients showed histological improvement of lupus nephropathy, 9 patients showed progression of lupus nephropathy and 7 of these who progressed, were started on intermittent IV CYC bolus.

The clinical follow up revealed that out of 46 patients, 21.7% patients went into complete remission, 58.6% patients remained under control with immunosuppressant medications, 10.8% patients had clinically active disease with normal renal function and 10.8% patients had adverse outcome. The adverse outcome included one patient developed chronic renal insufficiency, three (3) progressed to end stage renal diseases and one died. Five-year kidney survival was 93.5% and patient survival was 97.8%.

Although IV CYC treatment has improved the mortality and morbidity in lupus nephritis but severe adverse effect makes it less than optimal for long term therapy.

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