Objective To investigate the clinical and lung imaging features of patients with myeloperoxidase (MPO)- positive antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with lung involvement. Methods The general data, laboratory examinations, lung imaging changes, and complications of 59 patients with MPO-ANCA positive and diagnosed as AAV in the Department of Nephrology of No. 960 Hospital of Joint Logistics Support Force of PLA from Jan. 2014 to Dec. 2020 were retrospectively analyzed. According to the results of urine routine, serum creatinine, and estimated glomerular filtration rate, the patients were divided into simple lung injury group and lung and kidney combined injury group, and the above data of the 2 groups were compared and analyzed. Results Among the 59 patients, there were 27 males and 32 females, with an onset age of 24-93 (61.8±15.6) years old. There were 13 patients in the simple lung injury group and 46 in the lung and kidney combined injury group. There were no significant differences in age or gender of onset between the 2 groups (both P＞0.05). Compared with the simple lung injury group, the levels of hemoglobin ([85.13±23.79］g/L vs[116.38±23.99］g/L), albumin ([27.55±4.68］g/L vs[34.75±5.31］g/L), and the proportion of weight loss (4.3% [2/46］vs 30.8%[4/13］) in the lung and kidney combined injury group were significantly decreased, while the erythrocyte sedimentation rate ([73.60±35.04］mm/1 h vs[57.77±34.56］mm/1 h), C reactive protein (28.15[5.08, 103.00］mg/L vs 14.30[5.55, 58.70］mg/L), and the proportions of nausea/fatigue (37.0%[17/46］vs 7.7%[1/13］) and hypertension (87.0%[40/46］ vs 46.2%[6/13］) were significantly increased (all P＜0.05). All the 59 patients underwent chest computed tomography, and the most common imaging changes were grid changes, patch consolidation, and pleural thickening, but these characteristic changes had no significant differences between the 2 groups (all P＞0.05). Conclusion The lung imaging changes in MPOANCA positive AAV patients are varied, but the changes in the 2 groups are not specific. The lung and kidney combined injury group have more patients with hypertension and are prone to developing severe anemia and hypoproteinemia, suggesting a poor prognosis.