Plasmapheresis-induced remission in otherwise therapy-resistant FSGS.
We report an 8-year-old Caucasian boy who presented with steroid-resistant nephrotic syndrome. Renal biopsy showed the cellular variant of focal segmental glomerulosclerosis (FSGS). Within 1 year he received a series of therapies that have induced remission in other patients with this disease, all to no avail (conventional-dose cyclosporin A, methylprednisolone pulse therapy, high-dose cyclosporin A, and therapy with mycophenolate mofetil). He achieved remission after five sessions of plasma exchange. This case argues for aggressive therapy of resistant nephrotic syndrome in the native kidney. Plasma exchange should be considered as a possible rescue therapy arm in future study protocols.
Vécsei
A K
AK
St. Anna Kinderspital, Kinderspitalgasse 6, 1090 Vienna, Austria.
Müller
T
T
Schratzberger
E C
EC
Kircher
K
K
Regele
H
H
Arbeiter
K
K
Schroth
B
B
Aufricht
C
C
D002648:Child; D016572:Cyclosporine / Q000008:administration & dosage / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D004351:Drug Resistance; D005923:Glomerulosclerosis, Focal Segmental / Q000628:therapy*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D010956:Plasmapheresis; D012074:Remission Induction
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D009173:Mycophenolic Acid; D008775:Methylprednisolone