Background Magnetic resonance imaging (MRI) is the method of choice for evaluating systemic lupus erythematosus (SLE) patients with central nervous system (CNS) manifestations. Imaging findings in SLE patients are very diverse. Atrophy, diffuse or regional, enlarged ventricles and hyperintense white matter hyperintensities (WMH) have been described in variable frequency. However the prevalence and clinical significance of WMH in patients with disease onset before the age of 16 (jSLE) is still unknown.
Objectives The aim of this study was to analyze the frequency and clinical significance of WMH in both symptomatic and asymptomatic jSLE patients by a visual and semi-automatic segmentation method.
Methods We included consecutive cSLE patients followed in a cohort at the pediatric rheumatology unit at the State University of Campinas. All patients had disease-onset before the age of 16. Controls were matched for age, sex and demographic background. A complete clinical, laboratory, and neurologic evaluation was performed in all individuals. MRI scans were obtained through a standardized protocol (3Tessla Philips). FLAIR images were used for visual and semiautomatic volumetric measurements. We compared jSLE patients with controls using the 2-sample t-test. Analysis of variance was used to test for differences between groups. Linear regression was used to analyze the association between WMH and age, disease duration, disease activity [SLE Disease Activity Index (SLEDAI)], damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)]and total corticosteroid dose
Results The study included 51 patients (47 women) with mean age 16.96 years (SD ± 3.58) and 56 controls with mean age 17.25 years (SD ± 4.23). The white matter lesions were identified in 44 (86.2%) patients and in 4 (7.1%) controls (P<0.001). In jSLE predominantly subcortical lesions were identified (72%), followed by periventricular (12%), deep white matter (10%) and cortical lesions (6%). In the controls predominantly subcortical lesions (62%) were observed, followed by deep white matter (36%) and periventricular (2%) lesions. Both the number (n=1029 vs n=44) and volume of lesions (v=35796.4 vs v=1870.5 mm3) were significantly higher in jSLE patients when compared to controls (p<0.001). jSLE patients with WMH were significantly younger than controls with WMH (p<0.001). In jSLE, the presence of WMH was associated with the presence of cutaneous vasculitis (p<0.001). No other clinical and laboratory manifestation was associated with WMH in jSLE.
Conclusions The vast majority of jSLE patients presented WMH.The presence of small WMH was associated with vasculitis in jSLE patients, suggesting that these lesions are a result of the CNS involvement of the disease and not an incidental finding. Furthermore the quantitative MRI evaluation allowed us to determine better objective evaluation.
Disclosure of Interest A. Lapa Grant/Research support from: FAPESP 2010/13639-1, L. Ferretti Grant/Research support from: FAPESP 2010/11923-4, N. Sinicato Grant/Research support from: FAPESP 2010/13637-9, M. Postal Grant/Research support from: FAPESP 2009/10744-1, R. Marini: None Declared, F. Cendes: None Declared, S. Appenzeller Grant/Research support from: FAPESP 2008/02917-0; Conselho Nacional Pesquisa Desenvolvimento-Brasil CNPq (300447/2009-4)