Objective To summarize the clinical characteristics of patients with interstitial pneumonia with positive myeloperoxidase (MPO-IP). Methods The clinical data of 15 patients hospitalized with MPO-IP from June 2013 to January 2016 were analyzed retrospectively, including clinical manifestations, laboratory test, lung function test, chest high resolution computed tomography (HRCT) and management. Simultaneously, 11 patients with IPF were recruited as control. Results The onset age of MPO-IP was older than that in IPF patients (74.07±6.31 yearsvs. 66.73±6.80 years,P＜0.01). There was no manifestation of vasculitis in kidney in all included patients, and the differences were not significant in gender, smoking, cough and expectoration, wheezing, shortness of breath, HRCT manifestations, FVC%pred, TLCO%pred, PaO2 or PaCO2 between the two groups. In 15 MPO-IP patients, HRCT revealed 12 cases of usual interstitial pneumonitis (UIP), 2 cases of nonspecific interstitial pneumonia (NSIP), 1 case of organizing pneumonia (OP), and 5 patients with overlapped emphysema. Eleven IPF patients were all in UIP pattern and 4 with overlapped emphysema. One MPO-IP patients and 5 IPF patients had clubbed-fingers. The differences in clubbed-finger and treatment were significantly different between the two groups. Nine IPF patients received symptomatic treatment and7 MPO-IP patients were administered with glucocorticoids and immunosuppressants. In addition 2 MPO-IP patients were treated with pirfenidone. Conclusions MPO-IP patients have older onset age of disease and lower incidence of clubbed-finger than IPF patients. UIP is the main HRCT change both in MPO-IP and IPF. Beside glucocorticoid and immunosuppressants, pirfenidone is a choice and worthy of further research in management of MPO-IP.