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Cyclophosphamide therapy for lupus nephritis: poor renal survival in Arab children
1Department of Pediatrics, College of Medicine and King Khalid University Hospital, King Saud University, Riyadh 11461, Saudi Arabia. Asol333@Hotmail.com
Insights
High-dose intravenous cyclophosphamide showed short-term renal improvement in pediatric lupus nephritis. However, long-term data reveal significant progression to chronic renal failure and dialysis, questioning its sustained efficacy.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunosuppressive Therapy
Background:
- Limited published data exist on high-dose intravenous pulse cyclophosphamide for pediatric lupus nephritis.
- Long-term efficacy and safety of this treatment remain largely uncharacterized.
Purpose of the Study:
- To prospectively evaluate the 5-year clinical efficacy and safety of high-dose intravenous pulse cyclophosphamide in children with severe lupus nephritis.
Main Methods:
- Nine children with severe active lupus nephritis received monthly intravenous cyclophosphamide pulses (0.75-1 g/m(2)) for 6 months, followed by quarterly pulses for 36 months.
- Patients were followed prospectively for 5 years.
Main Results:
- Cyclophosphamide treatment demonstrated significant improvement in renal function during the therapy period.
- However, 56% of patients progressed to chronic renal failure, and 22% required dialysis within 2 years post-therapy.
- High initial serum creatinine and hypertension were associated with poorer long-term outcomes.
Conclusions:
- The studied regimen of intravenous high-dose pulse cyclophosphamide appears ineffective for long-term lupus nephritis management in children.
- This treatment approach may not prevent disease progression to end-stage renal disease, particularly in patients with severe initial presentation.
Abstract:
Despite its widespread use, there are only a few published studies of the use of intravenous high-dose pulse cyclophosphamide in lupus nephritis in children. There are few data about the long-term efficacy and safety of this form of therapy. This study evaluates the clinical efficacy of this regimen in children with severe lupus nephritis followed prospectively over a 5-year period. Nine children with severe active lupus nephritis were enrolled in a treatment regimen of monthly intravenous pulses of cyclophosphamide (0.75-1 g/m(2)) for 6 months and then every 3 months for a total of 36 months. Cyclophosphamide treatment was associated with significant improvement in renal function during treatment. However, data presented here show that 56% of the patients progressed to chronic renal failure and 22% required dialysis 2 years after discontinuation of cyclophosphamide therapy. Hence it seems that this regimen is not effective in our patients in the long term, especially patients who present with high serum creatinine and hypertension.
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