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Intravenous cyclophosphamide pulse therapy on children with severe active lupus nephritis
1Department of Pediatrics, National Taiwan University Hospital, Taipei, R.O.C.
Insights
This study shows that monthly intravenous cyclophosphamide (IVCY) therapy improved lupus nephritis in children. IVCY therapy enhanced kidney function and reduced disease markers while lowering corticosteroid dosage.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Lupus nephritis is a severe complication of systemic lupus erythematosus (SLE) in children.
- Treatment often involves corticosteroids, which can have significant side effects.
- Alternative or adjunctive therapies are needed to improve outcomes and reduce steroid toxicity.
Purpose of the Study:
- To evaluate the clinical efficacy and safety of monthly intravenous cyclophosphamide (IVCY) pulse therapy in children with active lupus nephritis.
- To assess the impact of IVCY on immunological markers and renal function.
- To determine if IVCY can reduce the required dosage of corticosteroids.
Main Methods:
- A preliminary study involving 13 children (mean age 14 years) with active lupus nephritis.
- Patients received monthly IVCY pulses in addition to regular corticosteroid therapy.
- Treatment criteria included biopsy-proven diffuse proliferative glomerulonephritis, steroid-resistant nephrotic syndrome, or severe steroid side effects.
Main Results:
- After 6 months, IVCY therapy showed significant improvements in serum C3, C4, and anti-DNA antibody titers (p < 0.01).
- Mean creatinine clearance significantly improved from 44.7 to 81.9 ml/min/1.73 m2 (p < 0.01).
- Mean prednisolone dosage was significantly reduced from 48.5 to 15.6 mg/day (p < 0.01).
Conclusions:
- Monthly IVCY pulse therapy is a potentially effective treatment for active lupus nephritis in children.
- IVCY therapy can improve immunological parameters and renal function.
- This approach may allow for a reduction in corticosteroid dosage, potentially mitigating side effects.
Abstract:
We conducted a preliminary study of monthly intravenous cyclophosphamide (IVCY) therapy on children with active lupus nephritis to evaluate the clinical efficacy and safety of IVCY pulse therapy. From June 1993 to May 1994, 13 patients (mean age: 14 +/- 0.9 years old; female: male = 11:2) with active lupus nephritis were treated with intermittent IVCY in addition to their regular corticosteroid therapy. Criteria for patients who received intermittent IVCY therapy were associated with any one of the following conditions: 1) with renal biopsy-proved diffuse proliferative glomerulonephritis; 2) with nephrotic syndrome and are inert to high dose of prednisolone therapy; and 3) with severe side effect of steroid therapy. Cyclophosphamide (CY) was administrated monthly for the first 6 doses, and at 2 to 3-month interval afterward for 2 years. The mean IVCY pulses per patient was 6.5 +/- 0.5 times. The initial dose of CY was 0.5 gm/mm2, then gradually increased to 1 gm/mm2 with a monthly increment of 0.25 gm/mm2. After 6 months, cyclophosphamide treatment was associated with significant improvement in mean levels of serum C3 (41.6 +/- 5.1 vs. 96.7 +/- 11.3 mg/dl), C4 (11.7 +/- 1.1 vs. 35.3 +/- 5.0 mg/dl) and anti-DNA titer (65.4 +/- 17.1 vs. 9.1 +/- 2.8 IU/ml) (all p < 0.01), despite a significant reduction in mean prednisolone dosage (48.5 +/- 4.8 vs. 15.6 +/- 3.2 mg/day; p < 0.01). The mean creatinine clearance also improved significantly from 44.7 +/- 5.7 to 81.9 +/- 2.7 ml/min/1.73 mm2 (p < 0.01) after 6 months of therapy.(ABSTRACT TRUNCATED AT 250 WORDS)