目的:系统性硬化症(SSc)不同亚型(dcSSc,弥漫性皮肤SSc;lcSSc,有限的皮肤SSc)尚不清楚。本研究旨在使用磁共振(MR)成像来表征不同亚型SSc患者的大脑结构和灌注变化。
方法:70例SSc患者(46.0±11.7岁,62名女性)和30名健康志愿者(44.8±13.7岁,招募24名女性),并进行了脑部MR成像和蒙特利尔认知评估(MoCA)测试。在T1加权图像上使用基于体素的形态计量学分析测量灰质(GM)体积。在动脉自旋标记图像上计算基于体素的局部脑血流量(CBF)。在dcSSc之间比较了MR成像的脑结构和灌注测量值,lcSSc和健康受试者使用单向ANOVA。还分析了临床特征与MR成像测量之间的相关性。
结果:与健康志愿者相比,dcSSc患者海马旁区域的GM体积显着减少(簇p<0.01,FWE校正)。然而,SSc患者,特别是lcSSc患者,小脑显示CBF升高,脑岛,大脑皮层,和皮质下结构(区域分析:所有p<0.05;基于体素的分析:聚类p<0.01,FWE校正)。此外,改良Rodnan皮肤评分(mRSS)的临床特征(r值范围为-0.29至-0.45),MoCA评分(r=0.40)和抗核抗体(ANA)阳性(r=-0.33)与某些地区的CBF显着相关(均p<0.05)。
结论:脑受累的表现因SSc的不同亚型而异。此外,严重皮肤硬化可能表明SSc患者脑受累的风险较高.
OBJECTIVE: The characteristics of brain impairment in different subtypes of systemic sclerosis (SSc) (dcSSc, diffuse cutaneous SSc; lcSSc, limited cutaneous SSc) remain unclear. This study aimed to characterize cerebral structure and perfusion changes in different subtype of SSc patients using magnetic resonance (MR) imaging.
METHODS: Seventy SSc patients (46.0 ± 11.7 years, 62 females) and 30 healthy volunteers (44.8 ± 13.7 years, 24 females) were recruited and underwent brain MR imaging and Montreal Cognitive Assessment (MoCA) test. Gray matter (GM) volumes were measured using voxel-based morphometry analysis on T1-weighted images. Voxel-based and regional cerebral blood flow (CBF) was calculated on arterial spin labelling images. The cerebral structural and perfusion measurements by MR imaging were compared among dcSSc, lcSSc and healthy subjects using one-way ANOVA. The correlations between clinical characteristics and MR imaging measurements were also analyzed.
RESULTS: The dcSSc patients exhibited a significant reduction in GM volume in the para-hippocampal region (cluster p < 0.01, FWE corrected) compared with healthy volunteers. Whereas, SSc patients, particularly lcSSc patients, showed elevated CBF in cerebellum, insula, cerebral cortex, and subcortical structures (regional analyses: all p < 0.05; voxel-based analyses: cluster p < 0.01, FWE corrected). Furthermore, clinical characteristics of modified Rodnan skin score (mRSS) (r value ranged from -0.29 to -0.45), MoCA scores (r = 0.40) and antinuclear antibody (ANA) positivity (r=-0.33) were significantly associated with CBF in some regions (all p < 0.05).
CONCLUSIONS: The manifestations of brain involvement vary among different subtypes of SSc. In addition, severe skin sclerosis may indicate higher risk of brain involvement in SSc patients.