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Pulse cyclophosphamide for steroid-resistant focal segmental glomerulosclerosis
W P Rennert1, U K Kala, D Jacobs
1Department of Pediatrics, Chris Hani Baragwanath Hospital, University of the Witwatersrand, Johannesburg, South Africa.
Pediatric Nephrology (Berlin, Germany)
|May 6, 1999
Summary
Intravenous pulse cyclophosphamide (IVCP) offers a promising adjunctive therapy for patients with steroid-resistant focal segmental glomerulosclerosis (FSGS). This treatment achieved sustained remission in secondary steroid-resistant FSGS and showed benefits in primary cases without adverse effects.
Area of Science:
- Nephrology
- Immunosuppressive Therapy
- Glomerular Diseases
Background:
- Focal segmental glomerulosclerosis (FSGS) is a leading cause of kidney disease.
- Steroid resistance is a significant challenge in managing FSGS, often leading to poor outcomes.
- Limited treatment options exist for patients with steroid-resistant FSGS.
Purpose of the Study:
- To evaluate the efficacy and safety of intravenous pulse cyclophosphamide (IVCP) as an adjunctive treatment for steroid-resistant FSGS.
- To assess the response rates in both primary and secondary forms of steroid-resistant FSGS.
- To determine the tolerability of IVCP in this patient population.
Main Methods:
- Ten patients with biopsy-proven steroid-resistant FSGS received monthly IVCP (500 mg/m2) for six months.
- Concurrent oral prednisone was administered with a specific tapering schedule over 12 months.
- Patients were categorized into primary (initial steroid resistance) and secondary (relapsing and then steroid-resistant) groups.
Main Results:
- All five patients with secondary steroid-resistant FSGS achieved sustained remission.
- In the primary steroid-resistant group, two patients achieved sustained remission, one had a partial response, and two showed no response.
- No adverse effects related to IVCP were reported by any of the patients.
Conclusions:
- IVCP is an effective adjunctive therapy for steroid-resistant FSGS, particularly beneficial for patients with secondary steroid resistance.
- The treatment demonstrated a favorable safety profile.
- Further research is warranted to optimize IVCP protocols for FSGS management.