Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups.

Lupus

PubMedID: 28134038

Lopes SR, Gormezano NW, Gomes RC, Aikawa NE, Pereira RM, Terreri MT, Magalhães CS, Ferreira JC, Okuda EM, Sakamoto AP, Sallum AM, Appenzeller S, Ferriani VP, Barbosa CM, Lotufo S, Jesus AA, Andrade LE, Campos LM, Bonfá E, Silva CA, Brazilian Childhood-onset Systemic Lupus Erythematosus Group. Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups. Lupus. 2017;961203317690616.
OBJECTIVE
The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (=6 and <12 years) and group C adolescent (=12 and <18 years).

METHODS
An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients.

RESULTS
Group A had 39 (4%), B 395 (47%) and C 413 (49%).Median disease duration was significantly higher in group A compared to groups B and C (8. 3 (0. 1-23. 4) vs 6. 2 (0-17) vs 3. 3 (0-14. 6) years, p??0. 05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p?=?0. 028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity.

CONCLUSIONS
This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes.This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.