Abstract： Objective To investigate the changes of major causes of death of patients with systemic lupus erythematosus (SLE).Methods Death cases with SLE from January 1986 to May 2011 in Peking Union Medical College Hospital were retrospectively analyzed.Results Out of 3554 patients with SLE,252 patients died,including 223 women and 29 men.The mortality rate was 7.2％ among female and 6.2％ among male,the overall mortality rate was 7.1％.The mortality rate in SLE patients had dropped steadily in the past 25 years,but there was a mild increase of mortality in 2006-2011 compared with that in 2001-2005 (5.7％vs 5.3％ ).In addition to infection,neuropsychiatric lupus and lupus nephritis had become the most common causes of death in SLE patients during the past 25 years.Furthermore,diffuse alveolar hemorrhage,severe pulmonary hypertension,coronary heart disease,thrombocytopenia,interstitial lung disease,lupus pneumonia,gastrointestinal hemorrhage, intestinal obstruction and multiple organ failure were the common causes of death,accounting for 4.4％,4.4％,3.2％,2.8％,2.4％,2.0％,2.0％,1.2％ and 1.2％ of all the death cases respectively.From 1986 to 2005,infection,neuropsychiatric lupus and lupus nephritis were the most common causes of death in patients with SLE,whereas the cases dying from lupus nephritis had decreased obviously and severe pulmonary hypertension had become the third most frequent causes of death during the past 5years.From 1986 to 1990,lupus nephritis,infection and neuropsychiatric lupus accounted for 31.4％,25.7％and 25.7％ of death cases respectively.From 1991 to 1995,lupus nephritis,infection and neuropsychiatric lupus accounted for 27.6％,24.1％ and 24.1％ respectively.From 1996 to 2000,infection,neuropsychiatric lupus and lupus nephritis took up 31.6％,21.1％ and 15.8％ respectively.From 2001 to 2005,infection,neuropsychiatric lupus and lupus nephritis took up 34.9％,20.6％ and 7.9％ respectively.From 2006 to 2011,infection, neuropsychiatric lupus and pulmonary hypertension accounted for 60.3％, 11.8％ and 7.4％ respectively.The mortality in the first year was the highest in the whole disease course,accounting for 32.5％ of patients.Deaths caused by neuropsychiatric lupus and infection happened most frequently during the first year,accounting for 41.9％ and 32.9％,whereas deaths caused by lupus nephritis occurred most frequently 10 years later,accounting for 32.3％.Age and gender had significant association with the major causes of death.The male patients took up 50.0％ of the total patients dying from coronary heart disease,in which 75.0％ of patients were older than 50 years.Conclusion Infection,neuropsychiatric lupus and lupus nephritis are the three most common causes of death in SLE patients fron 1986 to 2005.Severe pulmonary hypertension has become the third most frequent causes of death during the past 5 years instead of lupus nephritis.Severe infection has increased significantly and has been the leading cause of death in SLE patients in recent 5 years.