【Abstract】 Objective To retrospectively analyze the clinical features of ANCA associated vasculitis with pulmonary damage in elderly patients in an attempt to raise the awareness of this disease. Methods Clinical data of ANCA associated vasculitis with pulmonary damage in elderly patients (≥60yr) were retrospectively analyzed. Results The average age of the 11 elderly patients was 75.9 (60～89) years, and all had chronic underlying diseases. The median interval between onset and the diagnosis was 4 months (2 weeks～1 year). The common symptoms of vasculitis were cough and expectoration (90.9%), dyspnea or exertion intolerance (63.6%), hemoptysis (36.4%), and fever (45.5%). The most common imaging manifestations were interstitial pulmonary lesions (91.9%), which were found to be evident interstitial pulmonary fibrosis in 8 cases. Two cases displayed multiple large patches of exudative shadow, indicating diffuse alveolar hemorrhage. One case had nodule lesions. Renal damage was not detected at presentation in 8 cases (72.7%). Diagnosis was confirmed by serum ANCA assay and tissue biopsy. Although the majority achieved clinical remission after immunosuppressive therapy, 5 cases (45.5%) died of respiratory failure or severe infections within the induction therapy period or during follow-up. Conclusion Pulmonary involvement in patients with ANCA associated vasculitis was common and may be the first presentation. The symptoms and radiological manifestations of pulmonary involvement were variable, and interstitial pulmonary lesions were notable. Early diagnosis and appropriate treatment may improve the prognosis.