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Pulse cyclophosphamide therapy in steroid resistant nephrotic syndrome associated with mesangial proliferative
1Associate Professor &Consultant Pediatric Nephrologist, King Khalid University Hospital &King Saud University, Riyadh, Saudi Arabia.
Insights
Intravenous pulse cyclophosphamide (IVCP) effectively treats steroid-resistant mesangial proliferative glomerulonephritis (MesGN) in children. Most patients responded well, achieving steroid responsiveness and remission with minimal side effects.
Area of Science:
- Pediatric Nephrology
- Immunosuppressive Therapy
Background:
- Mesangial proliferative glomerulonephritis (MesGN) frequently presents as steroid-resistant in pediatric patients.
- Limited effective treatment options exist for steroid-resistant MesGN, necessitating alternative therapeutic strategies.
Purpose of the Study:
- To evaluate the efficacy and safety of intravenous pulse cyclophosphamide (IVCP) combined with oral prednisone in children with steroid-resistant MesGN.
- To assess long-term outcomes, including steroid responsiveness, remission rates, and relapse incidence.
Main Methods:
- Ten children diagnosed with steroid-resistant MesGN received monthly IVCP (500 mg/m² for six months) concurrently with oral prednisone.
- Prednisone dosage was initiated at 60 mg/m²/day for four weeks, followed by 40 mg/m²/alternate day for four weeks, then tapered over eight weeks.
- Patients were followed for four years post-treatment to monitor proteinuria, steroid dependence, and side effects.
Main Results:
- All ten patients achieved steroid responsiveness following IVCP therapy.
- Seven patients demonstrated an early response, with no proteinuria after the second IVCP dose.
- At four years, six patients achieved complete remission and were off steroids; four remained steroid-dependent. No significant adverse events were reported.
Conclusions:
- Intravenous pulse cyclophosphamide (IVCP) is a safe and effective treatment for pediatric steroid-resistant MesGN.
- IVCP can induce steroid responsiveness and achieve remission in a significant proportion of children with this condition.
- Further research is warranted to optimize treatment protocols and long-term management strategies.
Abstract:
High proportion of patients with mesangial proliferative glomerulonephritis (MesGN) is steroid resistant. We report the response of ten children with steroid resistant MesGN treated with intravenous pulse cyclophosphamide (IVCP) together with oral prednisone. All patients were treated with pulse IVCP 500 mg/m 2 per month for six months; oral prednisolone was given concurrently at a dose of 60 mg/m 2 per day for four weeks, then 40 mg/m 2 /per alternate day for another four weeks, which was tapered over the following eight weeks. All the patients were followed up for four years after treatment. All the patients became steroid responsive after the completion of IVCP therapy; nine patients were weaned of steroids and one patient could not. Seven patients showed early response to IVCP; they had no proteinuria after the 2nd dose of IVCP. Three patients had complete remission and never had any relapses over four years of observations. By the end of the observation period, there were six patients who achieved complete remission and they were off steroid, while four patients remained steroid dependent. None of the patients developed any significant side effects. We believe that IVCP is a safe and effective therapeutic modality in children with steroid resistant MesGN.
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