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Mortality in ANCA-associated vasculitis: ameta-analysis of observational studies
  1. Ju Ann Tan1,2,3,
  2. Natasha Dehghan2,
  3. Wenjia Chen4,
  4. Hui Xie1,5,
  5. John M Esdaile1,2,
  6. J Antonio Avina-Zubieta1,2
  1. 1 Arthritis Research Canada, Vancouver, British Columbia, Canada
  2. 2 Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada
  3. 3 Rheumatology, Western Health, Footscray, Victoria, Australia
  4. 4 Faculty of Pharmaceutical Sciences, University of British Columbia, Vancouver, British Columbia, Canada
  5. 5 Faculty of Health Sciences, Simon Fraser University, Vancouver, British Columbia, Canada
  1. Correspondence to Dr J Antonio Avina-Zubieta, Arthritis Research Canada, 5591 No. 3 Rd, Richmond, British Columbia V6X 2C7, Canada; azubieta{at}


Objective To determine the magnitude of all-cause mortality risk in patients with antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) compared with the general population through a meta-analysis of observational studies.

Methods We searched Medline and Embase databases from their inception to April 2015. Observational studies that met the following criteria were assessed by two researchers: (1) clearly defined AAV identified by either the American College of Rheumatology 1990 classification criteria or the 2012 Chapel Hill Consensus Conference disease definitions, and (2) reported standardised mortality ratios (SMR) and 95% CI. We calculated weighted-pooled summary estimates of SMRs (meta-SMRs) for all-cause mortality using random-effects model, tested for publication bias and heterogeneity.

Results Ten studies met the inclusion criteria, comprising 3338 patients with AAV enrolled from 1966 to 2009, and a total of 1091 observed deaths. Overall, we found a 2.7-fold increased risk of death in patients with AAV when compared with the general population (meta-SMR: 2.71 (95% CI 2.26 to 3.24)). Analysis on studies that included only granulomatosis with polyangiitis cases also indicated a similar mortality risk (meta-SMR: 2.63 (95% CI 2.02 to 3.43)). There was no significant publication bias or small-study effect. Subgroup analyses showed that mortality risks were higher in older cohorts, with a trend towards improvement over time (ie, those with their midpoint of enrolment periods that were between 1980–1993 and 1994–1999, vs 2000–2005).

Conclusion Published data indicate there is a 2.7-fold increase in mortality among patients with AAV compared with the general population.

  • ANCA
  • vasculitis
  • mortality
  • meta-analysis
  • observational studies

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  • Contributors JAT and JAA-Z conceived and designed the study. JAT and ND screened titles and abstracts for inclusion and extracted the data. All authors were responsible for data analysis and interpretation. JAT drafted the manuscript. All authors contributed to critical review of the manuscript and have read and approved the final manuscript.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.