Abstract
Background
Pediatric Systemic Lupus Erythematosus (pSLE) is a severe autoimmune disease due to its serious multi-visceral disorders, its morbidity causing deleterious and sometimes permanent effects. The objective is to determine the prognostic predictive factors of death in pSLE.
Methods
This was a prospective, descriptive, multicentre study, including patients less than16 years of age with SLE according to the criteria SLE of the American College of Rheumatology over a period of 36 months (2015–2018) carried out in the CHU Nefissa Hamoud in Algiers. The number of deaths during this follow-up period was determined and compared with surviving patients in several parameters (demographic, clinical, laboratory, therapeutic and disease activity). Disease activity was estimated by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), the use of which has been validated in children and damage and sequelae were appreciated by the Systemic Lupus International Collaborating Clinics (SLICC).
Means, percentages and χ2 tests were specified. The multivariate analysis of logistic regression was used to determine prognostic factors associated with the outcome variable. P values <0.05 were considered statistically significant.
Results
83 patients were collected and divided into two groups: deceased and survivors in which different parameters were compared and analyzed. Results: Female: male ratio 1: 4.9, mean age at diagnosis 11.3 ± 3.62. Clinical involvements at diagnosis were: cutaneous (100%), hematological (79.5%), articular (65, 1%), renal (44, 6%), neurological (41%), hepato-digestive (41%), cardiac (27, 7%), pleuropulmonary (19, 3%) and ocular involvement (7, 2%). The mean of SLEDAI was 22.11 ± 11.87 [interquartile range 2–53]. The prevalence of death was 11% of cases with an average age of death of 11 years [interquartile range 5–15 years]. The comparison of the two groups demonstrated a significant association between death and the following parameters: renal and neurological damage respectively: ((P = 0.03), (P = 0.02)), macrophage activation syndrome (MAS) (P = 0.0002), infections (P = 0.027), disease activity (P = 0.006) and damage score (P = 0.001). The mean SLEDAI activity in deceased patients was 32 ± 11.9 [interquartile range 17–53], compared with surviving patients which was 20.9 ± 11.4 [interquartile range 2–51].
Multivariate analysis with logistic regression revealed two major predictors of death, namely neurological involvement (odds ratio = 6,093 95% confidence interval ((1,1 8 0 ∼ 31 446)) and macrophage activation syndrome. Adherence to treatment was a protective factor: ([0.016 (0.001–0.353)].
Conclusion
These results showed that pediatric systemic lupus erythematosus exhibits an aggressive and severe phenotype with an unpredictable course. Studies are needed in children in order to specify and develop prognostic predictive factors and to identify patients at high risk of early mortality in order to design early and effective care of this vulnerable entity of lupus disease.
Keywords
Child, Systemic Lupus Erythematosus, mortality
Acute pancreatitis and macrophage activation syndrome in pediatric systemic lupus erythematosus: case-based review
