Introduction: Five- and ten-year survival of patients with systemic lupus erythematosus (SLE) increased to ˃90% within the last 50 years. Causes of death and survival are important indicators of treatment efficacy in SLE patients. No data are available on causes of death and survival of SLE patients in Croatia and neighboring countries. Hypothesis: Frequencies of categories of causes of death differ between early and late death. Aim: Analysis of disease characteristics and causes of death of patients deceased within the 2002-2011 period, and analysis of survival of patients diagnosed over the same period. Patients and methods: Characteristics of deceased patients fulfilling ≥4 classification criteria of the American Colege of Rheumatology (ACR) were analyzed. Demographics, classification criteria, damage and causes of death were compared between patients deceased early and late in their disease course. Survival analysis was performed in an inception cohort of patients diagnosed within 2002-2011. Results: 90 deceased patients were identified. Patients deceased earlier were diagnosed at a later age. The most frequent classification criteria were antinuclear antibodies, immunological and hematological disorder, with no difference between patients deceased early and late. At time of death the damage index was 4.9±3.2. The most frequent damage categories were musculoskeletal, cardiovascular and neuropsychiatric damage. Causes of death were: cardiovascular diseases (40%), infections (33%), active SLE (29%), malignant disease (17%) and other causes (11%). There was no difference between the frequencies of causes of early and late death, except for stroke, which caused only late death. Five- and ten-year survival in the inception cohort (185 non-deceased, 28 deceased patients) was 91% and 80.5%, respectively. Diagnosis at a later age, neuropsychiatric and renal disorder, as well as serositis were identified as predictors of death. Conclusion: No difference was observed in the frequencies of causes of early and late death, with the exception of stroke which only caused late death. The five-year survival is similar to survival observed in developed countries, while ten-year survival is unexpectedly lower.