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Plasma exchange improves the glomerulonephritis of systemic lupus erythematosus in selected pediatric patients
1Department of Pediatrics, Cedars-Sinai Medical Center, Los Angeles, California.
Insights
Plasma exchange (PE) offers a safe and effective treatment for children with severe lupus nephritis unresponsive to other therapies. This approach led to sustained remission in most patients, improving kidney function and nephrotic syndrome.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Systemic lupus erythematosus (SLE) can cause severe glomerulonephritis in children.
- Standard therapies like steroids and cytotoxic drugs are not always effective.
- Severe cases often involve nephrotic syndrome, renal failure, anemia, and leukopenia.
Purpose of the Study:
- To evaluate the efficacy of short-course plasma exchange (PE) followed by prednisone in children with severe SLE nephritis.
- To assess the long-term outcomes of this therapeutic approach.
Main Methods:
- Six children with severe SLE glomerulonephritis received PE and tapering prednisone.
- Patients had previously shown poor response to pulse methylprednisolone and/or cytotoxic drugs.
- Renal biopsies were analyzed, and patient responses were monitored for over a year.
Main Results:
- PE induced rapid and sustained remission of renal failure in 3/3 patients.
- Five of six patients achieved complete and sustained remission of nephrotic syndrome.
- Anemia and leukopenia also improved with PE.
- Histological findings included a high frequency of WHO Type V and IV lupus nephritis.
Conclusions:
- Plasma exchange is a safe and effective option for severe pediatric SLE nephritis unresponsive to conventional therapy.
- PE can induce long-term remission of renal failure and nephrotic syndrome.
- This treatment may be particularly beneficial for patients with specific lupus nephritis subtypes.
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.