Background: Takayasu arteritis (TA) is a large-vessel vasculitis that can involve pulmonary arteries (PAs). We studied multiple clinical characteristics related to pulmonary artery involvement (PAI) in TA patients.Methods: We enrolled 216 patients with TA from a large prospective cohort. PAI was assessed in each patient based on data from magnetic resonance angiography/computed tomography angiography. Pulmonary hypertension, cardiac function, and pulmonary parenchymal abnormalities were evaluated further in patients with PAI based on echocardiography, New York Heart Association Functional Classification and pulmonary computed tomography, respectively. These abnormalities related to PAI were followed up to evaluate treatment effects.Results: PAI was detected in 56/216 (25.93%) patients, which involved the pulmonary trunk, main PAs and small vessels in the lungs. Among patients with PAI, 28 (50%) patients were accompanied by pulmonary hypertension, which was graded as ‘severe’ in 9 (16.07%), ‘moderate’ in 10 (17.86%) and mild in 9 (16.07%). Forty (71.43%) patients had cardiac insufficiency (IV: 6, 10.71%; III: 20, 35.71%; II: 14, 25.00%). Furthermore, 21 (37.50%) patients presented with abnormal parenchymal features in the area corresponding to PAI (e.g., the mosaic sign, infarction, bronchiectasis). During follow-up, two patients died due to abrupt pulmonary thrombosis. In the remaining patients, the abnormalities mentioned above improved partially after routine treatment.Conclusions: PAI is very common in TA patients. PAI can cause pulmonary hypertension, cardiac insufficiency and pulmonary parenchymal lesions, which worsen the prognosis.