OBJECTIVES
We compared survival and causes of death in Western Australian (WA) anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and polyarteritis nodosa (PAN) patients with controls and the WA population.
METHODS
In this data linkage study, we identified patients with incident AAV/PAN and age, sex and temporally matched controls 1980 - 2014 from the WA Rheumatic Disease Epidemiological Registry. Survival analyses and time-varying analyses were performed.
RESULTS
614 patients with incident AAV/PAN were compared with 6672 controls; 229 AAV/PAN patients died over 5277 person years of follow-up and 1009 controls died over 73835 person years.Survival was reduced in patients with AAV/PAN compared with matched controls (hazard ratio (HR) 3.5 (95% confidence interval (CI) 3.1, 4.1)), and matched WA population rates (standardised mortality ratio (SMR) 3.3 (95%CI 2.9, 3.8)). Greatest excess mortality in AAV/PAN patients was observed in the first year after diagnosis and remained higher than controls throughout follow-up. Greater excess mortality was observed in patients >60 years at diagnosis.In cause-specific analyses, mortality HR for vasculitis, infection, non-infective respiratory disease were greatest early after diagnosis and remained persistently elevated. The HR for malignancy and cerebrovascular disease related deaths increased during follow-up, and were constant for ischaemic heart disease (IHD) related deaths.
CONCLUSION
Mortality was increased in AAV/PAN patients compared with controls, with patients older at diagnosis at greater risk. These findings provide mortality risk for AAV/PAN in an Australian population, highlighting key contributors to mortality at different time periods over follow-up and potential areas of focus for reducing mortality.