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Long-term patient survival in ANCA-associated vasculitis

Flossmann, Oliver; Berden, Annelies; de Groot, Kirsten; Hagen, Chris; Harper, Lorraine; Heijl, Caroline LU ; Höglund, Peter LU ; Jayne, David; Luqmani, Raashid and Mahr, Alfred, et al. (2011) In Annals of the Rheumatic Diseases 70(3). p.488-494
Abstract
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... (More)
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. (Less)
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Contribution to journal
publication status
published
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in
Annals of the Rheumatic Diseases
volume
70
issue
3
pages
488 - 494
publisher
British Medical Association
external identifiers
  • wos:000286927800014
  • scopus:79951518112
ISSN
1468-2060
DOI
10.1136/ard.2010.137778
language
English
LU publication?
yes
id
3fa26551-91f8-417d-8cf6-99c30beb11e4 (old id 1876627)
date added to LUP
2011-04-01 11:16:13
date last changed
2017-11-12 03:49:45
@article{3fa26551-91f8-417d-8cf6-99c30beb11e4,
  abstract     = {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 &lt;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.},
  author       = {Flossmann, Oliver and Berden, Annelies and de Groot, Kirsten and Hagen, Chris and Harper, Lorraine and Heijl, Caroline and Höglund, Peter and Jayne, David and Luqmani, Raashid and Mahr, Alfred and Mukhtyar, Chetan and Pusey, Charles and Rasmussen, Niels and Stegeman, Coen and Walsh, Michael and Westman, Kerstin},
  issn         = {1468-2060},
  language     = {eng},
  number       = {3},
  pages        = {488--494},
  publisher    = {British Medical Association},
  series       = {Annals of the Rheumatic Diseases},
  title        = {Long-term patient survival in ANCA-associated vasculitis},
  url          = {http://dx.doi.org/10.1136/ard.2010.137778},
  volume       = {70},
  year         = {2011},
}