title: Outcome analysis of patients with vasculitis associated with antineutrophil cytoplasmic antibodies.
abstract: BACKGROUND
  Objective scoring systems of disease activity and disease-associated damage have proven useful in the management of patients with systemic vasculitis.
  
  
  METHODS
  We used the recently designed Birmingham vasculitis activity score (BVAS; maximum score 63) and vasculitis damage index (VDI; maximum score 59) to assess initial activity and long-term damage, respectively, in ANCA positive patients from one center over a 3-year period. Thirty-two patients with ANCA vasculitis were identified and analyzed as an historic cohort. The median BVAS for all vasculitis patients at first presentation was 19 (range 6 - 36). Patients with Wegener's granulomatosis had a significantly higher total score and respiratory BVAS score compared to the 15 with microscopic polyangiitis. The majority of patients received standard cyclophosphamide/steroid treatment.
  
  
  RESULTS
  At the end of follow-up (mean 24.9 months), 4 patients had died; all patients had evidence of permanent organ damage. The median total VDI score at last follow-up was 4.0 (range 0-11), with no differences between patients with Wegener's granulomatosis and microscopic polyangiitis. The VDI was not associated with the number of relapses. A high initial BVAS was found to correlate with a later high vasculitis damage index (r = 0.56). Initial renal or respiratory involvement was also associated with longterm damage in the same organ system.
  
  
  CONCLUSIONS
  Although mortality from ANCA-associated vasculitis has decreased, morbidity remains a common problem. High early-disease activity may identify patients at high risk of long-term organ damage, allowing more effective individualized therapy. This hypothesis requires validation in a prospective, controlled study.
authors.affiliation: Department of Clinical Immunology, University Hospital of Groningen, The Netherlands.
authors.forename: F
authors.initials: F
authors.lastname: Brijker
authors.forename: C C
authors.initials: CC
authors.lastname: Magee
authors.forename: J W
authors.initials: JW
authors.lastname: Tervaert
authors.forename: S
authors.initials: S
authors.lastname: O'Neill
authors.forename: J J
authors.initials: JJ
authors.lastname: Walshe
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009017:Morbidity; D017063:Outcome Assessment, Health Care; D012720:Severity of Illness Index; D014657:Vasculitis / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology / Q000401:mortality
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide