Despite newer treatments with immunosuppressive agents, there still exists a considerable morbidity and mortality risk among patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). Since 1994 the European Vasculitis Society (EUVAS) has aimed for an improved outcome for patients with AAV by several prospective randomized clinical trials (RCTs). The aim for the present study was to further evaluate the long-term survival of patients with AAV included in seven RCTs conducted by the EUVAS, as well as to identify potential prognostic factors.
Long-term follow-up data were collected from questionnaires sent to the principal investigators of the original RCTs (1995-2012): MEPEX, NORAM, CYCAZAREM, CYCLOPS, IMPROVE, RITUXVAS and MYCYC, comprising 848 patients, all newly diagnosed with AAV. Relative survival estimates are presented for the study cohorts. Demographic, clinical and laboratory characteristics at trial entry were studied as potential prognostic factors in multivariable models.
478 (56%) patients had Granulomatosis with polyangiitis (GPA) and 370 (44%) Microscopic polyangiitis (MPA) with a mean age at diagnosis of 58 ± 14 years. Median follow-up time was 8 years (IQR: 2.9-13.6). During the observation period, there were 305 deaths and the main causes were infections (26%), cardiovascular disease (14%) and malignancies (13%). When compared to a matched cohort, (regarding country, age-group, and sex) from the background population there was 14.2% more deaths among our cohort of AAV patients at 5 years, 19.9% at 10 years, 28.8% at 15 years and 36.3% at 20 years. The excess mortality occurred in all age groups. Estimated median survival time (from diagnosis) was 17.8 years (95%CI: 15.7-20). Among variables measured at baseline, advanced age, male sex, low estimated glomerular filtration rate, and low platelet count were identified as predictors of death in a multivariate Cox model.
Patients with AAV still have an increased risk of mortality compared to the general population despite newer therapeutic regimens. Treatment complications and organ damage are the main causes of limited survival, and infections remain the leading cause of mortality among patients with AAV.

© The Author(s) 2023. Published by Oxford University Press on behalf of the ERA.