In Japan, the criteria for Wegener’s granulomatosis, MPA, and allergic granulomatous angiitis proposed by the Japan Research Committee of the Ministry of Health, Labour, and Welfare (JMHLW) [28] are still adopted for the diagnosis of diseases designated intractable by the Japanese government, with the name ‘Wegener’s granulomatosis’ replaced by GPA and ‘allergic granulomatous angiitis’ replaced by EGPA. Here, GYPA is linked to granulomatosis with polyangiitis.