title: Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study.
abstract: BACKGROUND
  Renal involvement is frequently present in antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitis and is an important cause of end-stage renal failure (ESRF).
  
  
  METHODS
  This retrospective, multicenter, sequential cohort study reports presenting features and outcome of 246 new patients diagnosed in London, UK, between 1995 and 2000.
  
  
  RESULTS
  Diagnostic subgroups were microscopic polyangiitis, 120 patients (49%); Wegener's granulomatosis (WG), 82 patients (33%); renal-limited vasculitis, 33 patients (13.5%); and Churg-Strauss angiitis, 11 patients (4.5%). Median age was 66 years, 57% were men, and median creatinine level at presentation was 3.87 mg/dL (342 micromol/L). ANCA was present in 92%. Cumulative patient survival at 1 and 5 years was 82% and 76%, respectively. Mortality was associated with age older than 60 years (P < 0.001), development of ESRF (P < 0.001), initial creatinine level greater than 2.26 mg/dL (200 micromol/L; P = 0.01), and sepsis (P < 0.048). ESRF occurred in 68 patients (28%), of whom 47% died. Fifty-six patients who presented with a creatinine level greater than 5.65 mg/dL (500 micromol/L) survived, and 31 patients (55%) achieved dialysis independence. Relapse occurred in 34% after a median of 13 months and was more common in patients with WG (P = 0.048) and proteinase 3-ANCA (P = 0.034). Leukopenia occurred in 41% and was associated with sepsis (P < 0.001).
  
  
  CONCLUSIONS
  Mortality and morbidity of ANCA-associated systemic vasculitis are improving compared with previous series, but remain high. Renal vasculitis often affects older patients, who have a particularly poor outcome. Early diagnosis improves outcome. Leukopenia, caused by immunosuppressive therapy, should be avoided because of the close association with sepsis and death.
authors.affiliation: Department of Medicine, Addenbrookes's Hospital, Cambs, UK. tonybooth1@yahoo.co.uk
authors.forename: Anthony D
authors.initials: AD
authors.lastname: Booth
authors.forename: Mike K
authors.initials: MK
authors.lastname: Almond
authors.forename: Aine
authors.initials: A
authors.lastname: Burns
authors.forename: Peter
authors.initials: P
authors.lastname: Ellis
authors.forename: Gill
authors.initials: G
authors.lastname: Gaskin
authors.forename: Guy H
authors.initials: GH
authors.lastname: Neild
authors.forename: Martin
authors.initials: M
authors.lastname: Plaisance
authors.forename: Charles D
authors.initials: CD
authors.lastname: Pusey
authors.forename: David R W
authors.initials: DR
authors.lastname: Jayne
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood* / Q000276:immunology; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology; D003404:Creatinine / Q000097:blood; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D007674:Kidney Diseases / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology; D007676:Kidney Failure, Chronic / Q000453:epidemiology / Q000209:etiology; D007970:Leukopenia / Q000139:chemically induced / Q000453:epidemiology; D015993:Life Tables; D008131:London / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D018805:Sepsis / Q000453:epidemiology / Q000209:etiology; D016019:Survival Analysis; D016896:Treatment Outcome; D014657:Vasculitis / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003404:Creatinine