The prospect of evaluating LAMP-2-ANCA for direct role(s) in the pathogenesis of renal disease associated with vasculitis or as a biomarker of glomerular damage (13) is inviting, particularly in children with AAV that, compared to adult-onset disease, present with more severe disease involving multiple organs (19, 26) and more than half of patients experience kidney damage early in disease course (27). This evidence concerns the gene LAMP2 and anti-neutrophil cytoplasmic antibody-associated vasculitis.