Background/Purpose: The CNS involvement in cSLE has been described to occur from 25-90% in childhood-onset systemic lupus erythematosus (cSLE). The aim of the study was to investigate the progression of brain and corpus callosum atrophy in cSLE patients.

Methods: We included consecutive cSLE patients followed in the pediatric rheumatology unit at the State University of Campinas. All patients had 2 magnetic resonance imaging (MRI) done during the follow-up period. MRI scans were obtained through a standardized protocol. Volumes ≤2 standard deviation (SD) from the means of controls were considered abnormal. Neurological manifestations were analyzed according to the ACR classification criteria. SLE patients were further assessed for clinical and laboratory SLE manifestations, disease activity [SLE Disease Activity Index (SLEDAI)], damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)] and current and cumulative drug exposures. Non-parametric-tests were used for statistical analysis.

Results: We included 44 cSLE patients (mean age 17.1±3.8 years). The control group consisted of 66 healthy volunteers (mean age 17.3±4.4 years) (p=0.15). At study entry, we observed smaller cerebral (mean volume 1073.1±123.8 cm³) and corpus callosum (CC) (mean volume 11.5 ±1.7 cm³) volumes in cSLE patients when compared to controls [mean cerebral volume 1182.9±118.9 cm³; p<0.001 (mean difference (MD): -1139.0 cm³; 95%CI 1114.1-1163.9)] and mean CC volume 13.6±2.1; cm³ p<0.001 (MD -12.8 cm³; 95%CI 12.4-13.2). Cerebral volumes were associated with anxiety (p=0.016), depression (p=0.002), antinuclear antibody (p=0.026) and anti-Sm antibody (p=0.013) in cSLE. CC volumes were associated with psychosis (p=0.039), acute confusional state (p=0.035) and low complement levels (p=0.01). After a mean follow-up mean period of 20.7±9.1 months we observed significant reduction in cerebral volume [mean 1060.6±128.9 cm³; p<0.001 (MD -1066.8 cm³; 95%CI: 1040.2-1093.5)] and CC volumes [mean volume 11.3±1.3 cm³; p<0.001 (MD-11.4 cm³; 95%CI: 11.1-11.7)] in cSLE patients. Reduction of cerebral volume was associated with corticosteroid dose per kilogram (p=0.035) and reduction of CC volume with nephritis (p=0.033) and low complement (p=0.046). Cerebral and CC volumes were stable in controls over time (p=0.3). Cerebral atrophy was observed in 9 (20.4%) cSLE patients and 1 (1.5%) controls at study entry and in 10 (22.7%) cSLE patients during the follow-up period (p=0.62). CC atrophy was observed in 9 (20.4%) cSLE patients and 1(1.5%) controls at study entry and in 10 (22.7%) cSLE patients during the follow-up period (p=0.62).  Cerebral atrophy was associated with anxiety (p=0.008), depression (p=0.014) and anti-Sm antibody (p=0.04) and CC atrophy with low complement levels (p=0.002) in cSLE. 

Conclusion: We observe progression of cerebral and CC volume loss in cSLE patients, although the number of patients with atrophy did not increase. Corticosteroid dose and disease activity (nephritis and low complement) were variables associated with progression of volume loss in cSLE.

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/longitudinal-analyses-of-progression-of-brain-athrophy-in-childhood-onset-systemic-lupus-erythematosus/