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Abstract Introduction Wegener granulomatosis (WG) appears with clinical symptoms, including recurrent respiratory infection, renal manifestations, and nonspecific systemic symptoms. Objective To study the clinical manifestations of WG in Iranian ethnicities, and data on 164 patients were recorded from 2013 to 2018. Methods The data included demographics, symptoms, and the Birmingham Vasculitis Activity Score (BVAS). The symptoms involved the following sites: the nose, sinus, glottis, ears, lungs, kidneys, eyes, central nervous system, mucous membranes, skin, heart, stomach, intestine, as well as general symptoms. The clinical manifestations of nine ethnicities were analyzed. Results In total, 48% of the patients were male and 51% were female, with a median age of 51 years. The BVAS was of 15.4, the sites most involved were the sinus (n =155), nose (n = 126), lungs (n = 125), and ears (n =107). Gastrointestinal (n = 14) and cardiac (n = 7) involvement were less common. Among the patients, 48.17% were Persian, 13.41% were Azari, 11.17% were Gilaki, 11.17% were Kurd, and 10.9% were Lor. Conclusion Our findings indicated that the sinus, nose, lungs, and ears were the sites most involved, and gastrointestinal and cardiac involvement were less common. In the present study, involvement of the upper and lower respiratory tract was higher than that reported in Western and Asian case series. Moreover, we report for the first time that, in all patients with ear involvement, the left ear was the first to be affected. The clinical manifestations among Iranian ethnicities were not different, and the Gilaki ethnicity had the highest BVAS, mostly because the weather was humid; therefore, in Iran, in areas with humidity, the rate of the disease was higher. (WG infection 16 201 2018 demographics BVAS. . (BVAS) glottis kidneys eyes system membranes skin heart stomach intestine analyzed total 48 female 5 years 154 15 4 15.4 n =155, 155 =155 , =155) 126, 126 126) 125, 125 125) =107. 107 =107 =107) 14 7 common 4817 17 48.17 Persian 1341 13 41 13.41 Azari 1117 11 11.17 Kurd 109 10 9 10.9 Lor series Moreover affected different humid therefore Iran humidity 1 20 (BVAS 15. =15 12 =10 481 48.1 134 13.4 111 11.1 10. 2 =1 48. 13. 11.