Long-term follow-up of patients with ANCA-associated vasculitis

This thesis is based on long-term follow-up results from two cohorts of patients with ANCA-associated vasculitis; one cohort with 535 patients originally included in 4 European randomized clinical trials (papers I and II) and one Swedish population-based cohort including 195 patients (papers III and IV). Two areas are covered in the two cohorts, respectively; the assessment of mortality, prognostic factors and causes of death in the two cohorts (paper I and III) and the assessment of malignancy risk compared with a matched general population (papers II and IV).
Results
Paper I: 1-, 2-, and 5-year survival was 88%, 85% and 78%. Predictors of death were age, gender, kidney function and disease activity at presentation. Main causes of death were active vasculitis and infection during the first year, and after the first year, malignancy and cardiovascular disease. Paper II: Higher risk for malignancy at all sites and non-melanoma skin cancer. Paper III: 1-, 2-, 5-, and 10-year survival was 87%, 82%, 70% and 55%, respectively. Predictors of death were age, gender, kidney function and organ involvement at diagnosis. Main causes of death were active vasculitis and infection during the first year, and after the first year, malignancy and cardiovascular disease. Paper IV: Higher risk for malignancy at all sites, non-melanoma skin cancer, bladder cancer and pancreatic cancer.
Conclusions: The mortality and malignancy risks in patients treated for AAV with current treatment protocols are higher than in the general population. However, the mortality in the group of patients with AAV without gastrointestinal, cardiovascular, or renal involvement at presentation is not significantly increased compared with the general population.