Department of Neurology, Affiliated Aerospace Hospital of Zunyi Medical University, Zunyi, Guizhou, China.
Department of Neurology, People's Hospital of Wuchuan County, Zunyi, Guizhou, China.
Front Immunol. 2024 Jul 31;15:1437848. doi: 10.3389/fimmu.2024.1437848. eCollection 2024.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogeneous but treatable immune-mediated neuropathy. Ofatumumab (OFA) is a fully human anti-CD20 monoclonal antibody that has shown promising efficacy in central demyelinating diseases, such as multiple sclerosis (MS). However, there is a lack of studies on the usage of OFA in peripheral demyelinating diseases, particularly CIDP. A case of relapsed and refractory CIDP with an ineffective response to conventional immunotherapy and intolerance to rituximab (RTX) but a positive response to subcutaneous injections of OFA is presented.
The patient, a 46-year-old man diagnosed with CIDP, received high-dose intravenous methylprednisolone, intravenous immunoglobulin (IVIG), and plasma exchange(PE) during the acute phase of the disease, and long-term oral administration of prednisone, azathioprine (AZA), and mycophenolate mofetil (MMF) during the remission phase. However, the patient suffered six relapses over a five-year period, and because of these, along with an ineffective response to conventional immunotherapy, and intolerance to RTX, subcutaneous injections of OFA were selected as a prophylactic treatment against relapses. After a total of six injections of OFA, CD19+B cells were substantially depleted. The patient has been followed for more than 23 months without relapse.
This case demonstrates the effectiveness and good tolerability of OFA in the treatment of relapsed and refractory CIDP. Further studies are needed to investigate the efficacy and safety of OFA in patients with relapsed and refractory CIDP, especially in those who have shown an ineffective response to conventional immunotherapy and are intolerant to RTX.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种异质性但可治疗的免疫介导的神经病。奥法妥珠单抗（OFA）是一种完全人源抗 CD20 单克隆抗体，在中枢脱髓鞘疾病（如多发性硬化症（MS））中显示出良好的疗效。然而，关于 OFA 在周围脱髓鞘疾病，特别是 CIDP 中的应用，研究较少。本文报道了 1 例复发性和难治性 CIDP 病例，该患者对常规免疫疗法无效且对利妥昔单抗（RTX）不耐受，但对皮下注射 OFA 有反应。
患者为 46 岁男性，被诊断为 CIDP。在疾病急性期，他接受了大剂量静脉注射甲基泼尼松龙、静脉注射免疫球蛋白（IVIG）和血浆置换（PE），在缓解期，他接受了长期口服泼尼松、硫唑嘌呤（AZA）和吗替麦考酚酯（MMF）。然而，该患者在五年内经历了六次复发，由于这些复发以及对常规免疫疗法无效且对 RTX 不耐受，选择皮下注射 OFA 作为预防复发的治疗方法。接受总共六次 OFA 注射后，CD19+B 细胞大量耗竭。患者在无复发的情况下接受了超过 23 个月的随访。
本病例表明 OFA 治疗复发性和难治性 CIDP 的有效性和良好耐受性。需要进一步研究来评估 OFA 在复发性和难治性 CIDP 患者中的疗效和安全性，特别是在对常规免疫疗法无效且对 RTX 不耐受的患者中。
