title: Kidney transplantation for primary focal segmental glomerulosclerosis: outcomes and response to therapy for recurrence.
abstract: BACKGROUND
  For a subset of adults and children with primary focal segmental glomerulosclerosis (FSGS), proteinuria and renal dysfunction recur after kidney transplantation (KTx). Predicting recurrence and response to plasmapheresis (PP) or other interventions remains problematic.
  
  
  METHODS
  The prevalence, recurrence rate, outcomes, and treatment responses of patients with FSGS were determined among 1573 KTx recipients. Although 5.0% carried some diagnosis of FSGS, only 1.9% (n=30) met strict diagnostic criteria for primary FSGS including biopsy-proven FSGS, lack of secondary factors, negative family history, and progression to end-stage renal disease within 10 years.
  
  
  RESULTS
  Of these, 47% had recurrent FSGS compared with 8% of those not meeting strict criteria (P<0.001). Recurrence was more common in children compared with adults (86% vs. 35%, P=0.01). Graft survival was lower for recipients with primary FSGS compared with all others and inferior graft survival was attributable to recurrent FSGS. Fourteen patients received PP preemptively (pre-KTx) or therapeutically (post-KTx) for recurrent disease. Four pediatric patients additionally received anti-CD20 (rituximab) therapy. Of the different treatment approaches, only PP combined with rituximab was associated with prolonged remission of proteinuria.
  
  
  CONCLUSIONS
  The results indicate that patients at high risk for FSGS recurrence can be identified and may benefit from carefully planned peritransplant interventions.
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN 55905, USA. hickson.latonya@mayo.edu
authors.forename: Latonya J
authors.initials: LJ
authors.lastname: Hickson
authors.forename: Manish
authors.initials: M
authors.lastname: Gera
authors.forename: Hatem
authors.initials: H
authors.lastname: Amer
authors.forename: Corey W
authors.initials: CW
authors.lastname: Iqbal
authors.forename: Therese B
authors.initials: TB
authors.lastname: Moore
authors.forename: Dawn S
authors.initials: DS
authors.lastname: Milliner
authors.forename: Fernando G
authors.initials: FG
authors.lastname: Cosio
authors.forename: Timothy S
authors.initials: TS
authors.lastname: Larson
authors.forename: Mark D
authors.initials: MD
authors.lastname: Stegall
authors.forename: Michael B
authors.initials: MB
authors.lastname: Ishitani
authors.forename: James M
authors.initials: JM
authors.lastname: Gloor
authors.forename: Matthew D
authors.initials: MD
authors.lastname: Griffin
mesh_terms: D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D002648:Child; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000517:prevention & control / Q000601:surgery*; D006085:Graft Survival; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation / Q000276:immunology / Q000401:mortality / Q000502:physiology*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D000069283:Rituximab; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab