Complete remission of post-transplant FSGS recurrence by long-term plasmapheresis.
Focal segmental glomerulosclerosis (FSGS) is known to recur in approximately 30% of renal allografts with graft loss in about half of these cases. The exact etiology remains unclear, though a putative circulating permeability factor or loss of inhibitory substances is being discussed. Different therapeutic approaches have been used. We report on a 10-year-old Arabian boy with a recurrence of FSGS immediately after transplantation. In addition to intensifying immunosuppressive therapy with high-dose cyclosporin A and cyclophosphamide, plasmapheresis was initiated and remission was achieved after 8 months. Three weeks after cessation of plasmapheresis a relapse occurred. Plasmapheresis was resumed and remission was achieved again after four additional sessions. The interval between plasmapheresis treatments was then gradually increased and fourteen months after transplantation plasmapheresis was stopped again. Since then (1.5 years after cessation of treatment) the patient has been in complete remission without any further episode of proteinuria. In conclusion, complete and sustained remission with stable renal function was achieved in our patient by long-term plasmapheresis in combination with intensified immunosuppression. Therefore, continuation of plasmapheresis treatment should be considered even in the situation of initial non-response.
Häffner
Karsten
K
Department of Pediatrics and Adolescent Medicine, Albert-Ludwigs-University, Mathildenstrasse 1, 79106 Freiburg, Germany.
Zimmerhackl
Lothar B
LB
von Schnakenburg
Christian
C
Brandis
Matthias
M
Pohl
Martin
M
D000284:Administration, Oral; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D016572:Cyclosporine / Q000008:administration & dosage / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D005923:Glomerulosclerosis, Focal Segmental / Q000601:surgery* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007275:Injections, Intravenous; D016030:Kidney Transplantation; D008297:Male; D010956:Plasmapheresis; D012008:Recurrence; D012074:Remission Induction; D012720:Severity of Illness Index; D013997:Time Factors
D007166:Immunosuppressive Agents; D016572:Cyclosporine; D003520:Cyclophosphamide