ObjectiveThe objective of this paper is to investigate survival and causes of death in a Danish lupus population.MethodsTwo hundred and fifteen SLE patients (94% Caucasians) were followed prospectively for up to 16 years. Thirty-eight patients died. Survival rate and causes of death were analysed.ResultsOverall standardized mortality ratio (SMR) was 2.2. Peak values were recorded for patients aged 20-29 (SMR 21.1). Cumulated survival rates at one, five, 10 and 15 years were 98.6%, 93.6%, 86.5% and 73.0%, respectively. The most common causes of death were cardiovascular events (32%), respiratory system disease (16%) and malignancies (13%). Deaths due to infections and active SLE were rare and predominated within the first seven years after diagnosis and before age 40, while cardiovascular deaths prevailed after 20 years' follow-up.ConclusionThis study shows that despite progress in lupus management, including direct access to specialized hospital care and increased use of hydroxychloroquine, mortality in lupus patients is still increased. Main causes of death were active disease and infections among the young and newly diagnosed, while cardiovascular deaths prevailed in longstanding disease.