RT Journal Article SR Electronic T1 Long-term patient survival in ANCA-associated vasculitis JF Annals of the Rheumatic Diseases JO Ann Rheum Dis FD BMJ Publishing Group Ltd and European League Against Rheumatism SP 488 OP 494 DO 10.1136/ard.2010.137778 VO 70 IS 3 A1 Oliver Flossmann A1 Annelies Berden A1 Kirsten de Groot A1 Chris Hagen A1 Lorraine Harper A1 Caroline Heijl A1 Peter Höglund A1 David Jayne A1 Raashid Luqmani A1 Alfred Mahr A1 Chetan Mukhtyar A1 Charles Pusey A1 Niels Rasmussen A1 Coen Stegeman A1 Michael Walsh A1 Kerstin Westman A1 for the European Vasculitis Study Group YR 2011 UL http://ard.bmj.com/content/70/3/488.abstract AB 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.