Sera LAMP-2-ANCA in chronic primary systemic vasculitis of childhood. (A) LAMP-2-ANCA concentration (y-axis; μg/mL) in sera from children and adolescents (x-axis) with systemic autoinflammatory disease (n = 18, SAID) and chronic primary systemic vasculitis (PSV) affecting small- (SVV, n = 53), medium- (MVV, n = 16) and large- (LVV, n = 21) sized blood vessels. Horizontal dotted line marks the threshold for LAMP-2-ANCA seropositivity in samples with greater than 1 μg/mL LAMP-2-ANCA. (B,C) LAMP-2-ANCA concentration (y-axis; μg/mL) in the pediatric chronic PSV cohort grouped (x-axis) based on (B) positivity for MPO-ANCA (n = 18), PR3-ANCA (n = 30), or neither MPO- or PR3-ANCA (negative, n = 35), and (C) biological sex. (D) Concentration of LAMP-2-ANCA (y-axis; μg/mL) in pediatric chronic PSV participants plotted against age of symptom onset (x-axis; years). (E) Age of symptom onset (y-axis; years) in LAMP-2-ANCA-seropositive and -seronegative patients with MVV and LVV (x-axis). Bars show means in (A–C), and medians in (E). LAMP-2-ANCA-seropositive (+) and -seronegative (−) samples indicated with closed and open circles, respectively. * Denotes statistical significance with p < 0.05, *** p < 0.001, **** p < 0.0001.