Surviving the butterfly and the wolf: mortality trends in systemic lupus erythematosus

Objective: To address changes in survival of patients with systemic lupus erythematosus (SLE) and to compare mortality statistics with associated disease specific as well as comorbid conditions. Methods: Review of the international literature on survival of patients with SLE. Results: During the first half of the 20th century, SLE was considered a rapidly progressive and almost invariably fatal disease. Since the 1950s, the estimated 5-year survival of SLE patients in developed countries rose from <50% to >95% and similar increases were seen in 10-year survival. Mortality rates of SLE patients, however, remain 3 times that of an age- and sex-matched population in most studies, indicating that death still occurs prematurely in a substantial portion of patients, albeit later in the disease course. This improved prognosis does not appear to have been shared equally by all racial/ethnic groups. This appears to be attributable more to socioeconomic and sociocultural factors than to true differences in disease manifestations. Along with the increased survival of SLE patients, there has been a change in the causes of death. Most notably, there has been a dramatic increase in the proportionate mortality from vascular disease, particularly accelerated atherosclerosis. Both disease and therapeutic modalities, in particular corticosteroids, appear to contribute to the high prevalence of coronary artery disease (CAD). Conclusions: Much progress has been made in improving the survival of SLE, but there is need for further improvement. Aggressive treatment of risk factors for CAD is advisable, but it remains to be assessed to what extent such interventions can further reduce mortality.