Plasma exchange improves the glomerulonephritis of systemic lupus erythematosus in selected pediatric patients

Abstract

The effects of short-course plasma exchange (PE) followed by tapering dose prednisone therapy was assessed in six children with systemic lupus erythematosus (SLE) and severe glomerulonephritis. All patients received pulse methylprednisolone therapy and three patients were treated with cytotoxic drugs prior to PE, but none had exhibited a good response. PE resulted in a rapid and sustained (greater than 1 year) remission of renal failure in the three patients with renal failure and severe glomerulonephritis. All six patients had severe nephrotic syndrome and five of six experienced a complete and sustained (greater than 1 year) remission post-PE (the sixth patient has greater than 4 month remission at the time of writing). Of interest was the high frequency of membranous [World Health Organization (WHO) Type V] and mixed membranous and diffuse proliferative SLE nephritis (WHO Type IV) on renal biopsy (4/6 patients). In addition, the severe anemia and leukopenia seen in most patients responded favorably to PE. Five of the six patients are currently managed on low-dose prednisone (0.25-0.5 mg/kg) every other day. One patient progressed to renal failure and dialysis more than 1 year post-PE. One patient required cytotoxic drug therapy post-PE (6 weeks). No significant complications were encountered; in fact, all patients eventually received their PE treatments as outpatients. We conclude that PE may provide a safe and effective therapeutic option for the treatment of severe progressive SLE nephritis in selected children who are unresponsive to steroid or cytotoxic drug therapy.