To assess the mortality profile of systemic lupus erythematosus (SLE) patients in France using multiple‐cause‐of‐death analysis.

Data were collected between 2000 and 2009 in the French Epidemiological Center for the Medical Causes of Death database, and death certificates issued upon the death of an adult for whom SLE was an underlying cause of death (UCD) or a non–underlying cause of death (NUCD) were evaluated using multiple‐cause‐of‐death analysis. Sex, age, sex ratio, standardized mortality rates, as well as frequency of the various causes of death were assessed, at both a national and a regional level. For the main causes of death, the observed number of deaths in relation to the expected number of deaths (O:E ratio) (standardized for age and sex) was calculated.

During the study period, 1,593 deaths related to SLE were identified. The mean ± SD age at death was 63.5 ± 18.4 years and the sex ratio (female:male) was 3.5. The mean standardized mortality rate was 3.2 per 1 million people (range 2.7–4.1). When SLE was the UCD (n = 637), the main NUCDs were cardiovascular diseases (49.5%), infectious diseases (24.5%), and renal failure (23.2%). When SLE was an NUCD (n = 956), the most common UCDs were cardiovascular diseases (35.7%), neoplasms (13.9%), and infectious diseases (10.3%). The overall O:E ratio was >1 for infectious and cardiovascular diseases and renal failure (especially among people <40 years of age for the latter 2 causes), but was <1 for neoplasms.

Cardiovascular disease is the leading cause of death associated with SLE in France.