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Extended report
Long-term patient survival in ANCA-associated vasculitis
  1. Oliver Flossmann1,
  2. Annelies Berden2,
  3. Kirsten de Groot3,
  4. Chris Hagen4,
  5. Lorraine Harper5,
  6. Caroline Heijl6,
  7. Peter Höglund6,
  8. David Jayne7,
  9. Raashid Luqmani8,
  10. Alfred Mahr9,
  11. Chetan Mukhtyar10,
  12. Charles Pusey11,
  13. Niels Rasmussen12,
  14. Coen Stegeman13,
  15. Michael Walsh14,
  16. Kerstin Westman6,
  17. for the European Vasculitis Study Group
  1. 1Renal Unit, Royal Berkshire Hospital, Reading, UK
  2. 2Leiden University Medical Center, Leiden, The Netherlands
  3. 3Klinikum Offenbach, Offenbach, Germany
  4. 4Meander Medical Center, Amersfoort, Netherlands
  5. 5College of Immunity and Infection, University of Birmingham, Birmingham, UK
  6. 6University of Lund, Skane University Hospital, Sweden
  7. 7Addenbrooke's Hospital, Cambridge, UK
  8. 8NIHR Musculoskeletal Biomedical Research Unit, Nuffield Orthopaedic Centre, University of Oxford, Oxford, UK
  9. 9Hôpital Cochin, Paris, France
  10. 10Norfolk and Norwich University Hospital, Norwich, UK
  11. 11Imperial College, London, UK
  12. 12Rigshospitalet, Copenhagen, Denmark
  13. 13University Hospital Groningen, Netherlands
  14. 14Department of Clinical Epidemiology and Biostatistics, McMaster University Hamilton, Canada
  1. Correspondence to Dr Oliver Flossmann, Royal Berkshire Hospital, Renal Unit, London Road, Reading, Berkshire RG1 5AN, UK; oflossmann{at}


Background Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.

Objective To describe the long-term patient survival and possible prognostic factors at presentation in an international, multicentre, prospectively recruited representative patient cohort who were treated according to strictly defined protocols at presentation and included the full spectrum of ANCA-associated vasculitis disease.

Methods Outcome data were collected for 535 patients who had been recruited at the time of diagnosis to four randomised controlled trials between 1995 and 2002. Trial eligibility was defined by disease severity and extent, covered the spectrum of severity of ANCA-associated vasculitis and used consistent diagnostic criteria. Demographic, clinical and laboratory parameters at trial entry were tested as potential prognostic factors in multivariable models.

Results The median duration of follow-up was 5.2 years and 133 (25%) deaths were recorded. Compared with an age- and sex-matched general population there was a mortality ratio of 2.6 (95% CI 2.2 to 3.1). Main causes of death within the first year were infection (48%) and active vasculitis (19%). After the first year the major causes of death were cardiovascular disease (26%), malignancy (22%) and infection (20%). Multivariable analysis showed an estimated glomerular filtration rate <15 ml/min, advancing age, higher Birmingham Vasculitis Activity Score, lower haemoglobin and higher white cell count were significant negative prognostic factors for patient survival.

Conclusion Patients with ANCA-associated vasculitis treated with conventional regimens are at increased risk of death compared with an age- and sex-matched population.

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  • Funding This study was supported by a grant from the European League Against Rheumatism and grants from Region Skåne, Sweden. OF and DJ were supported by the Cambridge Biomedical Research Centre. MW was supported by the Kidney Research Scientist Core Education and National Training (KRESCENT) Program and the Alberta Heritage Foundation for Medical Research.

    The funding sources had no involvement in the study's design, conduct or reporting.

  • Conflict of interest None.

  • Ethics approval This study was conducted with the approval of the West Midlands Multi-centre Research Ethics Committee (ref: MREC/98/7/37).

  • Provenance and peer review Not commissioned; externally peer reviewed