Krüger Christina, Pinnschmidt Hans, Wilmes Maximilian, Dargvainiene Justina, Leypoldt Frank, Seiler Alexander, Berg Daniela, Magnus Tim, Deb-Chatterji Milani
Department of Neurology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany.
Institute of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany.
Cells. 2025 Jun 24;14(13):966. doi: 10.3390/cells14130966.
Primary angiitis of the central nervous system (PACNS) is a rare vasculitis affecting CNS blood vessels, posing diagnostic challenges due to the limited specificity of the established diagnostic tools. Neurofilament light chain (NfL) might be a promising biomarker in PACNS.
NfL in serum and CSF was measured in 33 PACNS patients (25 active [aPACNS], 8 in remission [rPACNS]) enrolled between 2014 and 2022 and compared to controls (serum: = 303; CSF: = 68); Results: Serum NfL was significantly elevated in aPACNS (median: 45.77 pg/mL) versus rPACNS (6.68 pg/mL; < 0.001) and healthy controls (6.05 pg/mL; < 0.001). Similarly, CSF NfL was significantly elevated in aPACNS (median: 4914.58 pg/mL) compared to rPACNS (301.19 pg/mL; = 0.002) and controls (262.83 pg/mL; < 0.001). Serum and CSF NfL were significantly correlated (r = 0.90, < 0.001). Additionally, an association between elevated NfL and ischemic lesions was observed (serum: r = 0.59, = 0.006; CSF: r = 0.43, = 0.032). A subgroup analysis excluding stroke patients still revealed elevated NfL in 90% (CSF) and 50% (serum), with diminishing discriminatory power with older age.
NfL holds potential as a biomarker for PACNS, in particular in younger patients.
中枢神经系统原发性血管炎（PACNS）是一种罕见的影响中枢神经系统血管的血管炎，由于现有诊断工具的特异性有限，给诊断带来了挑战。神经丝轻链（NfL）可能是PACNS中有前景的生物标志物。
对2014年至2022年间纳入的33例PACNS患者（25例活动期[aPACNS]，8例缓解期[rPACNS]）的血清和脑脊液中的NfL进行检测，并与对照组（血清：n = 303；脑脊液：n = 68）进行比较；结果：与rPACNS（6.68 pg/mL；P < 0.001）和健康对照组（6.05 pg/mL；P < 0.001）相比，aPACNS患者的血清NfL显著升高（中位数：45.77 pg/mL）。同样，与rPACNS（301.19 pg/mL；P = 0.002）和对照组（262.83 pg/mL；P < 0.001）相比，aPACNS患者的脑脊液NfL也显著升高（中位数：4914.58 pg/mL）。血清和脑脊液NfL显著相关（r = 0.90，P < 0.001）。此外，观察到NfL升高与缺血性病变之间存在关联（血清：r = 0.59，P = 0.006；脑脊液：r = 0.43，P = 0.032）。排除中风患者的亚组分析仍显示，90%（脑脊液）和50%（血清）的患者NfL升高，且随着年龄增长，鉴别能力下降。
NfL有潜力作为PACNS的生物标志物，尤其是在年轻患者中。
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