Recently, the role of HMGB1 has been demonstrated in autoimmunity and chronic inflammatory diseases, especially those with renal manifestations such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, chronic kidney disease (CKD) and SLE [15,17,20-25]. This evidence concerns the gene HMGB1 and anti-neutrophil cytoplasmic antibody-associated vasculitis.