Recurrent nephrotic syndrome after transplantation: early treatment with plasmaphaeresis and cyclophosphamide.
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.
Cochat
P
P
Unité de Néphrologie Pédiatrique, Hôpital Edouard Herriot, Lyon, France.
Kassir
A
A
Colon
S
S
Glastre
C
C
Tourniaire
B
B
Parchoux
B
B
Martin
X
X
David
L
L
D000293:Adolescent; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D006973:Hypertension / Q000150:complications; D016030:Kidney Transplantation; D009404:Nephrotic Syndrome / Q000150:complications / Q000188:drug therapy* / Q000628:therapy*; D010956:Plasmapheresis; D011446:Prospective Studies; D011507:Proteinuria / Q000150:complications; D012008:Recurrence
D003520:Cyclophosphamide