Abstract:Although systemic lupus erythematosus (SLE) no longer has the very poor prognosis that it had 50 years ago, there remains a significant mortality. We were able to determine the causes of death in 27 of the 29 patients with SLE who died over the period 1985 to 1989. This represents one and five year mortality of six and 24% respectively. In common with other studies, sepsis was a major factor, being implicated in the deaths of 37% of our patients. However, sepsis (i) occurred almost exclusively in patients with active SLE; (ii) often occurred after prolonged hospitalisation; and (iii) was a terminal event in otherwise fatal SLE in several patients. Overall, active disease was determined to be a cause of death in 67% of the patients. An unexpected observation was the finding that active cardiopulmonary disease accounted for 37% of deaths. Although late mortality from degenerative vascular disease is being increasingly reported with the modern prolonged SLE survivorship, it was identified in only one patient. We conclude that active disease remains the most important factor in mortality in SLE. (Aust NZ J Med 1992; 22: 5–8