Neurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous System

Background: 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. Methods: NfL...

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Main Authors: Christina Krüger, Hans Pinnschmidt, Maximilian Wilmes, Justina Dargvainiene, Frank Leypoldt, Alexander Seiler, Daniela Berg, Tim Magnus, Milani Deb-Chatterji
Format: Article
Language:English
Published: MDPI AG 2025-06-01
Series:Cells
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Online Access:https://www.mdpi.com/2073-4409/14/13/966
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author Christina Krüger
Hans Pinnschmidt
Maximilian Wilmes
Justina Dargvainiene
Frank Leypoldt
Alexander Seiler
Daniela Berg
Tim Magnus
Milani Deb-Chatterji
author_facet Christina Krüger
Hans Pinnschmidt
Maximilian Wilmes
Justina Dargvainiene
Frank Leypoldt
Alexander Seiler
Daniela Berg
Tim Magnus
Milani Deb-Chatterji
author_sort Christina Krüger
collection DOAJ
description Background: 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. Methods: 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: <i>n</i> = 303; CSF: <i>n</i> = 68); Results: Serum NfL was significantly elevated in aPACNS (median: 45.77 pg/mL) versus rPACNS (6.68 pg/mL; <i>p</i> < 0.001) and healthy controls (6.05 pg/mL; <i>p</i> < 0.001). Similarly, CSF NfL was significantly elevated in aPACNS (median: 4914.58 pg/mL) compared to rPACNS (301.19 pg/mL; <i>p</i> = 0.002) and controls (262.83 pg/mL; <i>p</i> < 0.001). Serum and CSF NfL were significantly correlated (r = 0.90, <i>p</i> < 0.001). Additionally, an association between elevated NfL and ischemic lesions was observed (serum: r = 0.59, <i>p</i> = 0.006; CSF: r = 0.43, <i>p</i> = 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. Conclusions: NfL holds potential as a biomarker for PACNS, in particular in younger patients.
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spelling doaj-art-e06628f19b424f2e970d7b88c9ea2f762025-08-20T03:17:52ZengMDPI AGCells2073-44092025-06-01141396610.3390/cells14130966Neurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous SystemChristina Krüger0Hans Pinnschmidt1Maximilian Wilmes2Justina Dargvainiene3Frank Leypoldt4Alexander Seiler5Daniela Berg6Tim Magnus7Milani Deb-Chatterji8Department of Neurology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, GermanyInstitute of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, GermanyDepartment of Neurology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, GermanyNeuroimmunology Section, Institute of Clinical Chemistry, University Medical Center Schleswig-Holstein, 24105 Kiel, GermanyNeuroimmunology Section, Institute of Clinical Chemistry, University Medical Center Schleswig-Holstein, 24105 Kiel, GermanyDepartment of Neurology, University Medical Center Schleswig-Holstein, 24105 Kiel, GermanyDepartment of Neurology, University Medical Center Schleswig-Holstein, 24105 Kiel, GermanyDepartment of Neurology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, GermanyDepartment of Neurology, University Medical Center Schleswig-Holstein, 24105 Kiel, GermanyBackground: 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. Methods: 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: <i>n</i> = 303; CSF: <i>n</i> = 68); Results: Serum NfL was significantly elevated in aPACNS (median: 45.77 pg/mL) versus rPACNS (6.68 pg/mL; <i>p</i> < 0.001) and healthy controls (6.05 pg/mL; <i>p</i> < 0.001). Similarly, CSF NfL was significantly elevated in aPACNS (median: 4914.58 pg/mL) compared to rPACNS (301.19 pg/mL; <i>p</i> = 0.002) and controls (262.83 pg/mL; <i>p</i> < 0.001). Serum and CSF NfL were significantly correlated (r = 0.90, <i>p</i> < 0.001). Additionally, an association between elevated NfL and ischemic lesions was observed (serum: r = 0.59, <i>p</i> = 0.006; CSF: r = 0.43, <i>p</i> = 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. Conclusions: NfL holds potential as a biomarker for PACNS, in particular in younger patients.https://www.mdpi.com/2073-4409/14/13/966PACNSNfLvasculitisbiomarkerstrokeautoimmune diseases
spellingShingle Christina Krüger
Hans Pinnschmidt
Maximilian Wilmes
Justina Dargvainiene
Frank Leypoldt
Alexander Seiler
Daniela Berg
Tim Magnus
Milani Deb-Chatterji
Neurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous System
Cells
PACNS
NfL
vasculitis
biomarker
stroke
autoimmune diseases
title Neurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous System
title_full Neurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous System
title_fullStr Neurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous System
title_full_unstemmed Neurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous System
title_short Neurofilament Light Chain in Serum and CSF as a Potential Biomarker for Primary Angiitis of the Central Nervous System
title_sort neurofilament light chain in serum and csf as a potential biomarker for primary angiitis of the central nervous system
topic PACNS
NfL
vasculitis
biomarker
stroke
autoimmune diseases
url https://www.mdpi.com/2073-4409/14/13/966
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