Abstract: Objective To analyze the clinical characteristics of connective tissue disease(CTD) in patients with pulmonary embolism,and provide the basis for the early diagnosis and treatment. Methods We retrospectively reviewed the clinical data of 13 hospitalized patients with connective tissue disease and pulmonary embolism from January,2013 to December,2014.The clinical manifestations,imaging features,treatments and prognosis of these patients were described. Results Among the 13 patients,the rheumatoid arthritis(4 cases),systemic lupus erythematosus(3 cases),Sjogren’s syndrome(3 cases),primary antiphospholipid syndrome(1 case),autoimmune hemolytic anemia with antiphospholipid syndrome(1 case) and Polymyalgia Rheumatica(1 case) were combined.The main manifestations included dyspnea(9 cases),chest pain(6 cases),hemoptysis(4 cases) and fever(4 cases).Five cases were with extremity swelling,and 2 of them had unilateral lower limb swelling.CT pulmonary angiography performed in 11 cases showed that the complications included pulmonary artery trunk filling defect(1 case),lobar pulmonary artery filling defect(2 cases),segmental pulmonary artery filling defect(8 cases).Five cases underwent radionuclide pulmonary ventilation/perfusion(V/Q) examination,and the results showed that all of them were with pulmonary segment perfusion ventilation mismatch.Twelve cases were with other radiological changes,including effusion(5 cases),fibrosis(4 cases),bronchiectasis(4 cases),interstitial lung disease(2 cases) and pleural effusions/pleural thickening(6 cases).The transthoracic echocardiography inn 13 cases showed that 6 cases were with mild to severe pulmonary hypertension.Twelve cases underwent lower extremity venous ultrasound examination,and the results showed that 5 cases were with deep venous thrombosis,1 case with superficial venous thrombosis.All patients received anticoagulant therapy and the 30 days all-cause mortality was 0. Conclusion The symptoms such as dyspnea and chest imaging changes in the patients with connective tissue disease indicate the pulmonary embolism.