AAV = ANCA associated vasculitis, GPA = granulomatosis with polyangiitis, PR3 = Proteinase-3 ANCA, eGFR = estimated Glomerular Filtration Rate (CKD-EPI), ml/min/1.73 m2, BVAS = Birmingham Vasculitis Activity Score, CYC = Cyclophosphamide, RTX = Rituximab, ENT = Ear, Nose and Throat, 95% CI = 95% Confidence Interval. The gene discussed is PRTN3; the disease is granulomatosis with polyangiitis.