Kidney disease at diagnosis in paediatric-onset small vessel vasculitis stratified by ANCA status. A linear regression model was used to compare (A) kidney-specific disease activity, as measured by the renal pVAS (y-axis; median) (x-axis: ANCA-negative, n=43; MPO-ANCA, n=126; PR3-ANCA, n=232) and (B) kidney function, as measured by the eGFR (y-axis; median) (x-axis: MPO-ANCA, n=39; PR3-ANCA, n=84), at the time of diagnosis in children and adolescents with small vessel vasculitis. (C) An ordinal logistic regression model was used to compare the predicted probability (y-axis) of having (x-axis) normal kidney function (KDIGO stage 1, eGFR≥90), mildly reduced kidney function (KDIGO stage 2, 60≤eGFR≤89), moderately reduced kidney function (KDIGO stage 3, 30≤eGFR≤59), severely reduced kidney function (KDIGO stage 4, 15≤eGFR≤29) and kidney failure (KDIGO stage 5, eGFR<15) at diagnosis between MPO-ANCA (grey, n=39) and PR3-ANCA (black, n=84) seropositive AAV. All models were adjusted for age at diagnosis and sex as baseline variables. Data are presented as (A) boxplots with the median±IQR, (B) violin plots with the median (solid line) and IQR and (C) predicted probability±95% CI. AAV, ANCA-associated vasculitis; ANCA, anti-neutrophil cytoplasmic antibody; eGFR, estimated glomerular filtration rate; KDIGO, Kidney Disease Improving Global Outcomes; MPO, myeloperoxidase; pVAS, paediatric Vasculitis Activity Score.