Background ANCA- associated vasculitis (AAV) is a potentially life threatening condition requiring careful monitoring and balancing of treatment options to minimize morbidity and mortality.
Objectives To investigate the influence of baseline disease characteristics and induction therapy on the disease course and outcome in ANCA- associated vasculitis.
Methods Single centre longitudinal cohort study of all adult patients with an EMEA algorithm based diagnosis of AAV followed up to August 2016. Clinical data including disease activity (BVAS), ANCA type and level, treatment, relapses (BVAS >3) and organ damage (VDI) and other complications (e.g. infections) during the disease course were recorded. Predictors for ESRD, death, cancer and damage accrual were analysed by multivariate logistic regression presented as Odds Ratios (OR).
Results A total of 63 patients (59% male) (mean age at diagnosis 57 years, 59% with GPA, 24% MPA and 16% EGPA) were included. Fluorescence ANCA was positive in 92%, while 47% had MPO-ANCA and 43% PR3-ANCA. Induction therapy included corticosteroids (92%), Cyclophosphamide (57%), Rituximab (35%) and Plasmapheresis (6%). During 46 months of follow-up 34 patients (54%) experienced 71 relapses (rate 2.5/100 months) and 55 serious complications occurred (rate 2/100 months). Mean VDI at last follow-up was 2.1 with only 11 patients (17.4%) not developing organ damage. Averaged BVAS correlated with last VDI scores (Rs 0.25,p=0.012). Overall, 4 patients (6.3%) died, 19 (27%) developed renal insufficiency of which 2 (3.1%) required chronic dialysis, while 6 (9.5%) developed a new cancer. Age was an independent predictor (OR 1.09, p=0.05) for patient survival (95% and 91% at 1 and 5 years), but no effect was seen for baseline BVAS, gender, AAV or ANCA subtype (all p>0.1).
Conclusions While current treatment reduces the risk of death, AAV is still associated with a high rate of disease relapse, organ damage accrual and serious complications.
Disclosure of Interest None declared