Among AAVs, granulomatosis with polyangiitis (GPA) is characterized by granulomatous inflammation, usually involving the upper and lower respiratory tract, with frequent necrotizing glomerulonephritis, and with ANCA-targeting proteinase 3 (PR3) in most patients with active disease.1 Remission induction treatment of new-onset organ-threatening or life-threatening AAV usually combines glucocorticoids with cyclophosphamide or rituximab. This evidence concerns the gene PRTN3 and granulomatosis with polyangiitis.