Yang Meng-Ge, Xu Li, Ji Suqiong, Gao Huajie, Zhang Qing, Bu Bitao
Department of Neurology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, People's Republic of China.
Neuropsychiatr Dis Treat. 2022 Jun 16;18:1207-1217. doi: 10.2147/NDT.S361461. eCollection 2022.
To investigate the response of tacrolimus to chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies against paranodal proteins, including neurofascin-155 (NF155), contactin-1 (CNTN1) and contactin-associated protein 1 (Caspr1).
We retrospectively reviewed all CIDP patients who carried anti-NF155, CNTN1 and Caspr1 antibodies and were treated with tacrolimus at Tongji hospital from Jan 2018 to Apr 2021.
There were 58 patients with CIDP and only 9 patients had autoantibodies against paranodal proteins (17.2%). Five of the 9 patients received tacrolimus treatment with an initial dose of 2-3 mg once daily. One patient with anti-CNTN1 antibody started tacrolimus and corticosteroid treatment, at the first episode and eventually achieved full clinical remission without relapse. Four patients with anti-NF155 or -Caspr1 antibodies experienced relapse during corticosteroids tapering. Then, they were given oral tacrolimus and presented with clinical improvement. During follow-up, only one patient developed worsening weakness due to unreasonable tacrolimus discontinuation. Moreover, 3 patients were successfully withdrawn from corticosteroids and 2 patients took corticosteroids at low maintenance dose (10mg/d) after tacrolimus treatment. No severe adverse events were observed in all the patients.
Patients with autoantibodies against paranodal proteins had a better long-term outcome after adding tacrolimus. Combination therapy with corticosteroids and tacrolimus may be an effective therapeutic regimen.
探讨他克莫司对伴有抗结旁区蛋白自身抗体的慢性炎症性脱髓鞘性多发性神经病（CIDP）的疗效，这些抗结旁区蛋白自身抗体包括神经束膜蛋白-155（NF155）、接触蛋白-1（CNTN1）和接触蛋白相关蛋白1（Caspr1）。
我们回顾性分析了2018年1月至2021年4月在同济医院接受他克莫司治疗的所有携带抗NF155、CNTN1和Caspr1抗体的CIDP患者。
共有58例CIDP患者，其中仅9例患者存在抗结旁区蛋白自身抗体（17.2%）。9例患者中有5例接受了他克莫司治疗，初始剂量为每日一次2 - 3毫克。1例抗CNTN1抗体患者在首次发作时开始使用他克莫司和皮质类固醇治疗，最终实现完全临床缓解且无复发。4例抗NF155或 - Caspr1抗体患者在皮质类固醇减量过程中出现复发。随后，他们接受口服他克莫司治疗，临床症状改善。在随访期间，仅1例患者因不合理停用他克莫司导致肌无力加重。此外，3例患者成功停用皮质类固醇，2例患者在接受他克莫司治疗后以低维持剂量（10毫克/天）服用皮质类固醇。所有患者均未观察到严重不良事件。
添加他克莫司后，伴有抗结旁区蛋白自身抗体的患者长期预后较好。皮质类固醇与他克莫司联合治疗可能是一种有效的治疗方案。
