Abstract： Objective To investigate the potential chnical factors associated with the prognosis of sys-temic lupus erythematosus with pulmonary arterial hypertension patients. Methods By using the strategy of ret-respective cohort study, the research samples were the consecutive SLE-PAH inpatients chosen from Jan. 1997 to Jan 2008. The survival probabilities were estimated by Kaplan-Meier method. By adopting the Cox proportional hazard regression model to analyze the potential clinical factors associated with prognosis. Results 40 cases were chosen as a group for this study,among which 37 cases(92.5%)were completed the follow-up,while 7 (18.9%)cases ended with death(positive results). The follow-up ended in Jan. 2008. The follow-up lasted for (26.8 ±14.79) months. Diminishing LVEF(HR = 15.06,95% CI:1.778 ～ 127.64,P =0.013) ,NYHA clas-sification (HR = 11. 805,95 % CI : 1.628 ～ 85.61, P = 0.015) and treated with huge dose cyclophosphamide (HR =0.094,95% CI:0.014～0.65 ,P = 0.017)were associated with the prognosis from the COX multiplicity re-gression analysis. Conclusion The diminishing of the LVEF and worse cardiac function were positive factors to predict death. Treated with huge dose of CTX could improve the prognosis.