title: Pulse cyclophosphamide for steroid-resistant focal segmental glomerulosclerosis.
abstract: We report the response of ten patients (6 male, 4 female) with steroid-resistant focal segmental glomerulosclerosis (FSGS) to treatment with intravenous pulse cyclophosphamide (IVCP) together with oral prednisone. All patients had been treated with 60 mg/m2 oral prednisone daily for 2 months upon initial presentation. IVCP was given monthly at a dose of 500 mg/m2 over 6 months. Oral prednisone was given concurrently at 60 mg/m2 daily for 2 months and then on alternate days for 4 months, followed by 30 mg/m2 on alternate days for 6 months. Prednisone was then tapered monthly by 10 mg and finally discontinued. Five patients failed to respond to steroids from the onset and were considered as primary steroid resistant. Two of these patients achieved sustained remission after IVCP, one patient showed a partial response, with loss of edema, normalization of serum albumin, and persistent proteinuria, while two patients showed no response to IVCP. The other five patients had achieved remission after 2 months of daily prednisone at 60 mg/m2 upon initial presentation, but had suffered from more than three relapses per year and had eventually become steroid resistant. They were considered secondary steroid resistant. All five patients achieved sustained remission after IVCP. None of our patients suffered from adverse effects of IVCP. We suggest IVCP as an adjunctive therapy for steroid-resistant FSGS, particularly for patients with secondary steroid resistance.
authors.affiliation: Department of Pediatrics, Chris Hani Baragwanath Hospital, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: W P
authors.initials: WP
authors.lastname: Rennert
authors.forename: U K
authors.initials: UK
authors.lastname: Kala
authors.forename: D
authors.initials: D
authors.lastname: Jacobs
authors.forename: S
authors.initials: S
authors.lastname: Goetsch
authors.forename: S
authors.initials: S
authors.lastname: Verhaart
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D000477:Alkylating Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D004351:Drug Resistance; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy*; D006801:Humans; D007275:Injections, Intravenous; D008297:Male; D013256:Steroids
chemical_list: D000477:Alkylating Agents; D000893:Anti-Inflammatory Agents; D013256:Steroids; D003520:Cyclophosphamide