〔Abstract〕 To investigate the risk factors of primary Sjögren′s syndrome with pulmonary arterial hypertension(pSS-PAH) for early diagnosis and treatment, a total of 438 patients with primary Sjögren′s syndrome(pSS) were selected. Patients were divided into two groups according to pulmonary arterial pressure detected by cardiac ultrasonography. The clinical and laboratory characteristics between the two groups were compared. Among 438 patients, 80 had PAH, the incidence rate was 18.3%.Age of pSS-PAH was 22～77(59.9±13.8) years old, and pulmonary arterial pressure was between 5.33～15.19 kPa. Multivariate logistic regression analysis suggested that interstitial pneumonia, age, and initial pulmonary symptoms were the risk factors for pSS-PAH. In these cases, cardiac ultrasonography should be performed as soon as possible, early diagnosis and treatment can improve the prognosis of patients.