128 Outcomes of 847 childhood-onset systemic lupus erythematousus patients in three age groups

Background and aims To assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease( Methods Observational cohort study of 10 Paediatric Rheumatology centres, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was 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 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, with higher mortality rate and neuropsychiatric/vascular/skin organ damages 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.