Recurrent nephrotic syndrome after transplantation: Early treatment with plasmaphaeresis and cyclophosphamide

Abstract

Steroid-resistant nephrotic syndrome (NS) with focal glomerulosclerosis (FGS) and its recurrence after transplantation are mainly seen in children. The recurrence rate approximates 30% and the graft loss is about half this. Several therapeutic regimens have been proposed, giving conflicting results. In an attempt to remove a putative circulating factor and inhibit its production by lymphocytes, three patients with biopsy-proven FGS in the native kidney were included in a prospective uncontrolled trial using early plasmaphaeresis followed by substitutive immunoglobulins in association with methylprednisolone pulses and cyclophosphamide instead of azathioprine over a 2-month period. The patients were girls, aged 6.5, 13.3 and 15.8 years, who received a cadaveric transplant; concomitant immunosuppression included prednisone and cyclosporine A. All three patients exhibited early recurrence of the NS and were treated 5-10 days after the onset of proteinuria. Rapid and sustained remission was achieved in all patients within 12-24 days on therapy. One patient experienced a late acute but steroid-sensitive rejection episode; another suffered from septic ankle arthritis as a complication of reinforced immunosuppression. The latter girl had a second late recurrence of proteinuria that was controlled within 7 weeks. With a 18- to 27-month follow-up, all three patients have normal renal function, normal blood pressure and no proteinuria. We conclude that intensive therapy using plasmaphaeresis, steroid pulses and cyclophosphamide over a 2-month period can induce complete remission in children with early recurrence of NS after transplantation.

An Important Problem: Posttransplant Focal Segmental Glomerulosclerosis Recurrence and Plasmapheresis

“…In several reports, recurrent FSGS has also been associated with an increased number of acute rejection episodes (22)(23)(24). Surprisingly, a higher number of acute rejections in the non-recurrent group were found when compared with the …”

Therapeutic plasma exchange for the treatment of pediatric renal diseases in 2013

“…TPE was performed in six of those patients, with five of the six achieving remission and more rapid remission achieved in patients who were treated in the first 48 h after diagnosis of relapse [47]. Cochat et al [49] reported similarly promising results in another case series of three pediatric patients with recurrent FSGS within 2 weeks of renal transplant; all patients were treated with TPE, and all achieved remission and had preserved renal function. Recurrence of FSGS is associated with worse graft outcomes, and achieving remission may improve graft survival [50].…”

“…Some investigators have suggested that FFP supplementation as part of replacement fluids be used in actively infected plasma exchange patients with IgG levels of <100 mg/dL [130]. In patients undergoing frequent plasma exchange, low-dose IVIG (100-150 mg/kg/dose) infusions have also been used to avoid hypogammaglobulinemia [49].…”

Therapeutic Approach to Focal and Segmental Glomerulosclerosis (FSGS) Recurrence in Kidney Transplant Recipients

“…In the case of recurrence treatments are not standardized, and show inconstant results. Most reports concern children and involve a small number of patients 15,[38][39][40][41][42][43][44][45][46] . Cyclosporine (CsA) has shown some degree of efficacy in pediatric subjects 43,44 .…”