Granulomatosis with polyangiitis (GPA) (prior known as Wegener’s granulomatosis) represents a rare autoimmune disease characterized by a systemic necrotizing granulomatous vasculitis of small- and medium-sized blood vessels in association with positive cytoplasmic-staining anti-neutrophil cytoplasmic antibodies (cANCA) [1]. The gene discussed is PRTN3; the disease is granulomatosis with polyangiitis.