All patients with IPF had been treated with antifibrotic agents (nintedanib n = 14 (300 mg/day n = 4, 200 mg/day n = 10) or pirfenidone n = 18 (1800 mg/day n = 3, 1200 mg/day n = 11, 600 mg/day n = 4)), whereas only one patient with MPO-ANCA nephritis had received antifibrotic therapy (nintedanib 300 mg/day) (Table 2). This evidence concerns the gene MPO and idiopathic pulmonary fibrosis.