title: Long-term plasmapheresis and protein A column treatment of recurrent FSGS.
abstract: Transient or intermittent plasmapheresis with concurrent immunosuppressive therapy is thought to be beneficial in the treatment of recurrent focal segmental glomerulosclerosis (FSGS) in the early post-transplant period. The results of long-term (6-year) plasmapheresis therapy, in a 9-year-old female with an immediate recurrence of FSGS [urinary protein/urinary creatinine (UP/UC)=17.7] after cadaveric renal transplant, are presented. A 4-week plasmapheresis course induced a decline in the proteinuria, but a relapse occurred after cessation of plasmapheresis. Addition of protein A column therapy led to a further decrease in the proteinuria, to a non-nephrotic range. Long-term control of the nephrotic syndrome was established using a chronic treatment regimen consisting of a single-volume plasmapheresis, followed by a protein A column treatment, performed on sequential days every 3-4 weeks. Mean UP/UC values decreased to 1.15+/-0.9. A course of cyclophosphamide was successfully used to control a worsening of proteinuria 4 years post transplant. Although sequential renal biopsies demonstrated progressive glomerular sclerosis, the patient's mean calculated creatinine clearance only modestly declined from 78.3 ml/min per 1.73 m2, at the time of transplantation, to 62.7 ml/min per 1.73 m2, 6 years later. This patient demonstrated dependence on plasmapheresis/protein A column therapy to maintain a clinical remission of her FSGS recurrence. While long-term plasmapheresis and protein A column therapy in combination with immunosuppressive therapy reversed the effects of uncontrolled nephrosis and possibly facilitated long-term renal allograft survival, the glomerular sclerosis continued to progress.
authors.affiliation: Department of Pediatrics, Section of Pediatric Nephrology, Lucile Salter Packard Children's Hospital, Stanford University, Stanford, CA 94305, USA. abelson@stanford.edu
authors.forename: A
authors.initials: A
authors.lastname: Belson
authors.forename: P D
authors.initials: PD
authors.lastname: Yorgin
authors.forename: A Y
authors.initials: AY
authors.lastname: Al-Uzri
authors.forename: O
authors.initials: O
authors.lastname: Salvatierra
authors.forename: J
authors.initials: J
authors.lastname: Higgins
authors.forename: S R
authors.initials: SR
authors.lastname: Alexander
mesh_terms: D002648:Child; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000628:therapy*; D006801:Humans; D016030:Kidney Transplantation; D010956:Plasmapheresis; D012008:Recurrence; D013205:Staphylococcal Protein A / Q000627:therapeutic use*; D013997:Time Factors
chemical_list: D013205:Staphylococcal Protein A