Llauradó A, García-Carmona C, Restrepo-Vera J L, Alemañ J, Salvadó M, Sanchez-Tejerina D, Sotoca J, Seoane J L, Lainez E, Gratacós-Viñola M, Vidal-Taboada J M, Fissolo N, Comabella M, Raguer N, Juntas-Morales R
Neuromuscular Diseases Unit, Department of Neurology, Vall d'Hebron University Hospital, Vall d'Hebron Research Institute, Barcelona, Spain; Department de Medicina, Universitat Autónoma de Barcelona, Barcelona, Spain.
Neuromuscular Diseases Unit, Department of Neurology, Vall d'Hebron University Hospital, Vall d'Hebron Research Institute, Barcelona, Spain; Department de Medicina, Universitat Autónoma de Barcelona, Barcelona, Spain.
J Neurol Sci. 2025 Mar 15;470:123397. doi: 10.1016/j.jns.2025.123397. Epub 2025 Jan 18.
The development of new biomarkers is essential to improve diagnostic accuracy and guide treatment decisions in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The aim of this study was to investigate the utility of the serum neurofilament light chain (sNfL) level as a marker for disability and response to immunomodulatory treatment in patients with CIDP.
This prospective, single-center, observational study included 38 patients with CIDP: 19 treatment-naive (CIDP-I) patients assessed before and after the initiation of immunomodulatory therapy and 19 stable patients on maintenance immunoglobulins (CIDP-M). Clinical scales (INCAT, I-RODS, MRC-SS and grip strength) were used to assess disability and treatment response. Nerve conduction study data were collected.
The median sNfL level (pg/mL) was greater in CIDP-I patients than in CIDP-M patients (23.4 vs. 7.7; p = 0.002). A reduction in sNfL levels was observed in CIDP-I patients after 5 months of immunomodulatory treatment (23.4 vs. 15.0; p = 0.001). sNfL levels were correlated with greater disability as assessed by the INCAT (p = 0.007), I-RODS (p = 0.004), and MRC-SS (p = 0.016) in treatment-naive patients but not in those receiving maintenance therapy. sNfL levels correlated with the average amplitude of the distal compound muscle action potential of the median, ulnar, peroneal and tibial nerves from the most affected limb (p = 0.043).
sNfL levels are significantly reduced in patients with CIDP who respond to immunomodulatory treatment and are positively correlated with disability. These findings highlight the utility of sNfL as a marker of disease activity and treatment response in patients with CIDP.
开发新的生物标志物对于提高慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）的诊断准确性和指导治疗决策至关重要。本研究的目的是探讨血清神经丝轻链（sNfL）水平作为CIDP患者残疾程度和免疫调节治疗反应标志物的效用。
这项前瞻性、单中心、观察性研究纳入了38例CIDP患者：19例初治患者（CIDP-I）在免疫调节治疗开始前后进行评估，19例接受维持性免疫球蛋白治疗的稳定患者（CIDP-M）。使用临床量表（INCAT、I-RODS、MRC-SS和握力）评估残疾程度和治疗反应。收集神经传导研究数据。
CIDP-I患者的sNfL水平中位数（pg/mL）高于CIDP-M患者（23.4对7.7；p = 0.002）。免疫调节治疗5个月后，CIDP-I患者的sNfL水平有所降低（23.4对15.0；p = 0.001）。在初治患者中，sNfL水平与INCAT（p = 0.007）、I-RODS（p = 0.004）和MRC-SS（p = 0.016）评估的更高残疾程度相关，但在接受维持治疗的患者中则不然。sNfL水平与最受累肢体的正中神经、尺神经、腓总神经和胫神经的远端复合肌肉动作电位平均波幅相关（p = 0.043）。
对免疫调节治疗有反应的CIDP患者的sNfL水平显著降低，且与残疾程度呈正相关。这些发现突出了sNfL作为CIDP患者疾病活动和治疗反应标志物的效用。
