【Abstract】 Objective To analyze the clinical and imaging characteristics of connective tissue disease (CTD) associated with interstitial lung disease (ILD) and pulmonary hypertension PH), improve clinical understanding of the disease and provide help for the early diagnosis and early intervention of the disease. Methods 278 CTDILD patients were included in the present study. The patients were divided into CTDILD group and CTDILDPH group. The clinical and imaging features were anylyzed. Results The incidence of secondary ILD of CTD was 189%, and the incidence of secondary PH of CTDILD was 162%. Prevalence of primary disease in patients with CTDILD included SLE (30%), RA (352%), pSS (189%), PM/DM (69%), MCTD (43%) and SSc (47%). The incidence of shortness of breath, dyspnea, and Raynaud's phenomenon of skin sclerosis of the CTDILDPH group were significantly higher than that in CTDILD group. ( P＜005). The positive rate of ANA antibody in CTDILDPH group was higher than that in CTDILD group (P＜005). FVC%pred and D1CO%pred of CTDILDPH group were significantly lower than that of CTDILD group( P＜005). The right ventricular diameter and right ventricular outflow tract diameter, pulmonary artery diameter of CTDILDPH group were significantly higher than that of CTDILD group (P＜005). Grid opacities, and pulmonary artery thickening and enlargement of the heart were significantly higher than those in group CTDILD. ( P＜005). Conclusion Patients with shortness of breath, dyspnea, Raynaud's phenomenon, skin sclerosis and ANA antibody positive need to improve the lung CT/HRCT and echocardiography examination. CT/HRCT scan showed mesh opacities, thickening of pulmonary artery, heart shadow increase and / or echocardiogram showed right ventricular diameter, right ventricular outflow tract diameter, pulmonary artery diameter increases need to consider the secondary PH, which should timely monitor pulmonary artery pressure and given clinical intervention. Pulmonary function in patients with CTDILD, suggested that FVC%pred D1CO%pred decreased significantly and should be taken PH.