BACKGROUND: To evaluate the mortality patterns of SLE and the associated risk factors in Koreans. METHODS: Using the National Health Insurance database spanning 2008 to 2018, incident cases of SLE in patients aged 10-79 years were included. We analysed the all-cause mortality and cause-specific mortality, stratifying by sex and age. The mortality rate (MR) was calculated as the number of deaths per 100 000 person-years (PYs). The causes of death were identified by the International Classification of Diseases, 10th Revision codes during hospitalisation or emergency visit prior to death. A generalised estimating equation model was employed for risk factor analysis. RESULTS: In total, 11 375 incident SLE cases among patients with an average age of 42.3±16.7 years were recruited (86.1% female). During 57 658 PYs, 728 deaths occurred (MR 1262.62/100 000 PYs). The MR among men (2718.86/100 000 PYs) exceeded that among women (1060.57/100 000 PYs). The leading causes of death were SLE-related conditions (381.56/100 000 PYs), cardiovascular disease (CVD) (202.92/100 000 PYs), cancer (175.17/100 000 PYs) and infection (143.95/100 000 PYs). Of the SLE-related mortality, the key risk factors were pulmonary complications, such as pulmonary alveolar haemorrhage (OR 9.93), pulmonary arterial hypertension (OR 3.77) and interstitial lung disease (OR 3.27). CONCLUSIONS: Among Korean patients with SLE, SLE-related conditions were the leading causes of mortality. However, CVD and cancer were also identified as the main causes of mortality. Furthermore, pulmonary manifestations were significantly associated with SLE-related mortality.