自身免疫性神经节病与 CIDP 的临床相关性：大型队列的纵向评估。
Clinical relevance of distinguishing autoimmune nodopathies from CIDP: longitudinal assessment in a large cohort.
机构信息
Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
Department of Neurology, Amsterdam Neuroscience, Amsterdam UMC, location AMC, Amsterdam, The Netherlands.
出版信息
J Neurol Neurosurg Psychiatry. 2023 Dec 14;95(1):52-60. doi: 10.1136/jnnp-2023-331378.
BACKGROUND
The aim of this study was to determine treatment response and whether it is associated with antibody titre change in patients with autoimmune nodopathy (AN) previously diagnosed as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and to compare clinical features and treatment response between AN and CIDP.
METHODS
Serum IgG antibodies to neurofascin-155 (NF155), contactin-1 (CNTN1) and contactin-associated protein 1 (CASPR1) were detected with cell-based assays in patients diagnosed with CIDP. Clinical improvement was determined using the modified Rankin scale, need for alternative and/or additional treatments and assessment of the treating neurologist.
RESULTS
We studied 401 patients diagnosed with CIDP and identified 21 patients with AN (10 anti-NF155, 6 anti-CNTN1, 4 anti-CASPR1 and 1 anti-NF155/anti-CASPR1 double positive). In patients with AN ataxia (68% vs 28%, p=0.001), cranial nerve involvement (34% vs 11%, p=0.012) and autonomic symptoms (47% vs 22%, p=0.025) were more frequently reported; patients with AN improved less often after intravenous immunoglobulin treatment (39% vs 80%, p=0.002) and required additional/alternative treatments more frequently (84% vs 34%, p<0.001), compared with patients with CIDP. Antibody titres decreased or became negative in patients improving on treatment. Treatment withdrawal was associated with a titre increase and clinical deterioration in four patients.
CONCLUSIONS
Distinguishing CIDP from AN is important, as patients with AN need a different treatment approach. Improvement and relapses were associated with changes in antibody titres, supporting the pathogenicity of these antibodies.
背景
本研究旨在确定自身免疫性神经节病（AN）患者的治疗反应及其与抗体滴度变化的关系，这些患者之前被诊断为慢性炎症性脱髓鞘性多发性神经病（CIDP），并比较 AN 和 CIDP 之间的临床特征和治疗反应。
方法
采用基于细胞的检测方法检测诊断为 CIDP 的患者血清中神经束蛋白-155（NF155）、接触蛋白-1（CNTN1）和接触蛋白相关蛋白 1（CASPR1）的 IgG 抗体。采用改良的 Rankin 量表、替代和/或额外治疗的需要以及治疗神经科医生的评估来确定临床改善。
结果
我们研究了 401 名诊断为 CIDP 的患者，发现了 21 名 AN 患者（10 名抗 NF155、6 名抗 CNTN1、4 名抗 CASPR1 和 1 名抗 NF155/抗 CASPR1 双阳性）。在 AN 患者中，共济失调（68%比 28%，p=0.001）、颅神经受累（34%比 11%，p=0.012）和自主神经症状（47%比 22%，p=0.025）更为常见；与 CIDP 患者相比，静脉注射免疫球蛋白治疗后改善较少（39%比 80%，p=0.002），需要额外/替代治疗的频率更高（84%比 34%，p<0.001）。在治疗有效的患者中，抗体滴度降低或转为阴性。在四名患者中，停用治疗与抗体滴度升高和临床恶化相关。
结论
区分 CIDP 和 AN 很重要，因为 AN 患者需要不同的治疗方法。改善和复发与抗体滴度的变化相关，支持这些抗体的致病性。
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