Background/Purpose:

Although regarded as a disease of adulthood, SLE is also seen in children, and is associated with an increased risk for aggressive clinical course and major organ damage in comparison to adults. However, mortality rates and causes of death are under reported in the pediatric population, so that most of our understanding derived from generalizations of the adult literature. In France, a previous retrospective multicenter survey conducted on a ten-year period (1996-2006) identified 12 deaths in pediatric SLE patients. A better knowledge of the causes of death and the related comorbidities is pivotal to improve strategies to prevent death in children suffering SLE. The aim of the MORTALU-Ped study was to analyze the mortality profile related to pediatric SLE in France using a multiple cause-of-death analysis.

Methods:

For the 2000-2011 period, data was collected from the database of the French Epidemiological Center of Medical Causes of Death (CepiDc, Inserm) corresponding to death certificates (DC) on which SLE was listed as an underlying or non-underlying cause of death  (ICD-10 code L93 or M32) were analyzed for all patients under the age of 18 years. Gender, age, sex-ratio, as well as the causes of death were assessed. 

Results:

Overall, 35 DC were identified, of which 5 were excluded from further analyses (Age at death < 1 year; diagnosis of neonatal lupus with congenital heart block n=3; polymalformative syndrome n=1; cerebral hemorrhage n=1). Among the 30 remaining DC, SLE was notified as an underlying cause in 24 (80 %) and as a non-underlying cause of death in 6 (20 %). Mean number of causes of death reported on DC was 3.7 [2; 6]. The patients had a median age at death was 15 years [2; 17] and a sex ratio of 3 (23 female and 7 male). The median number of annual death was 3 [0; 5], relatively stable over time during the study period. Deaths were distributed across 20 French departments, with 1 or 2 DC by department, except for n°78 (Yvelines, n=3) and n°97 (ovserseas separtments, n=6).

For half patients (n=15), at least one severe SLE manifestation was reported: neurologic (n=4), cardiac (n=4), nephritis (n=3), hematologic (n=3), pancreatic (n=2) and pulmonary (n=1). Severe infections was reported in 9/30, 2 in a context of aplasia. Reported pathogens were Streptococcus pneumoniae (n=2), methicillin-resistant Staphylococcus aureus (n=1), Gram negative bacteria (n=1), histoplasmosis (n=1), crytpococcosis (n=1). Other noticeable causes of death were pulmonary embolism (n=1) and associated severe autoimmune condition (autoimmune cirrhosis).

Conclusion:

To our knowledge, this is the first mortality study using a multiple cause-of-death analysis in pediatric SLE. This study shows the interest of such approach to collect a more important number of cases of death in the context of a rare condition such as pediatric SLE. In pediatric SLE patients, the causes leading to death seem to be dominated by severe disease manifestations as well as by severe and/or opportunistic infections and lower sex ratio compared to the living pediatric population suggests higher severity in young male patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/mortality-related-to-pediatric-systemic-lupus-erythematosus-a-multiple-cause-of-death-analysis-in-france/