title: Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression.
abstract: BACKGROUND
  Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that usually presents as rapidly progressive glomerulonephritis, often with pulmonary hemorrhage (the Goodpasture syndrome). It is reported that patients with severe renal failure do not generally recover renal function.
  
  
  OBJECTIVE
  To examine the long-term outcome of severe anti-GBM antibody disease.
  
  
  METHODS
  Retrospective review of patients treated for confirmed anti-GBM antibody disease over 25 years.
  
  
  METHODS
  A tertiary referral center in the United Kingdom.
  
  
  METHODS
  71 treated patients with anti-GBM antibody disease.
  
  
  METHODS
  All patients received plasma exchange, prednisolone, and cyclophosphamide.
  
  
  METHODS
  Patient and renal survival, renal histology, and antibody levels.
  
  
  RESULTS
  Patients who presented with a creatinine concentration less than 500 micromol/L (5.7 mg/dL) (n = 19) had 100% patient survival and 95% renal survival at 1 year and 84% patient survival and 74% renal survival at last follow-up. In patients who presented with a creatinine concentration of 500 micromol/L or more (>/=5.7 mg/dL) (n = 13) but did not require immediate dialysis, patient and renal survival were 83% and 82% at 1 year and 62% and 69% at last follow-up. In patients who presented with dialysis-dependent renal failure (n = 39), patient and renal survival were 65% and 8% at 1 year and 36% and 5% at last follow-up. All patients who required immediate dialysis and had 100% crescents on renal biopsy remained dialysis dependent.
  
  
  CONCLUSIONS
  Patients with the Goodpasture syndrome and severe renal failure should be considered for urgent immunosuppression therapy, including plasma exchange, to maximize the chance of renal recovery. Patients needing immediate dialysis are less likely to recover.
authors.affiliation: Renal Section, Imperial College School of Medicine, Hammersmith Hospital, Ducane Road, London W12 ONN, United Kingdom.
authors.forename: J B
authors.initials: JB
authors.lastname: Levy
authors.forename: A N
authors.initials: AN
authors.lastname: Turner
authors.forename: A J
authors.initials: AJ
authors.lastname: Rees
authors.forename: C D
authors.initials: CD
authors.lastname: Pusey
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019867:Anti-Glomerular Basement Membrane Disease / Q000097:blood / Q000150:complications / Q000276:immunology / Q000628:therapy*; D003131:Combined Modality Therapy; D003404:Creatinine / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011239:Prednisolone / Q000627:therapeutic use*; D012008:Recurrence; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D003404:Creatinine