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.

Efficacy of intravenous pulse cyclophosphamide treatment versus combination of intravenous dexamethasone and oral cyclophosphamide treatment in steroid-resistant nephrotic syndrome

“…Furthermore, the proportion of patients whose condition was initially steroid resistant was 36.7% in the study reported here compared with 75% in the previous report [11]. Since patients with an initially steroid-resistant condition are considered less likely to respond to treatment [7,8,11,17], the inclusion of more subjects with late resistance in our study here might have influenced the results.…”

“…While patients with MCD and late resistance are believed to respond satisfactorily to immunosuppressive therapy [7,22], we could not show a differential response based on histology or pattern of resistance, perhaps due to the small number of subjects. While there was a trend towards an earlier age of onset in patients showing complete or partial remission than in those with no response, differences between the groups were not significant.…”

“…Previous studies suggest that remission of proteinuria following i.v. treatment with cyclophosphamide usually occurs with 3-5 doses of the drug [7,8,11,15]. Similarly, studies with i.v.…”

“…The efficacy of i.v. treatment with cyclophosphamide in patients with lupus nephritis and other vasculitides has led to its use in patients with SRNS [7,8].…”

Cyclophosphamide in the treatment of focal segmental glomerulosclerosis

“…Although its natural course has been well described, its treatment is still a matter of debate (4)(5)(6)(7)(8)(9)(10)(11)(12). Prednisone is the initial treatment and its therapeutic response seems to be the best predictor of outcome.…”