关键词: chronic inflammatory demyelinating polyradiculoneuropathy efficacy ofatumumab refractory relapse

Mesh : Humans Male Middle Aged Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / drug therapy Antibodies, Monoclonal, Humanized / therapeutic use administration & dosage Treatment Outcome Recurrence

来  源:   DOI:10.3389/fimmu.2024.1437848   PDF(Pubmed)

Abstract:
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要:
慢性炎性脱髓鞘性多发性神经根神经病(CIDP)是一种异质性但可治疗的免疫介导性神经病。Ofatumumab(OFA)是一种完全人抗CD20单克隆抗体,在中枢脱髓鞘疾病中显示出有希望的疗效,如多发性硬化症(MS)。然而,缺乏OFA在外周脱髓鞘疾病中的应用研究,特别是CIDP。一例复发性和难治性CIDP,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呈阳性反应。
病人,一名46岁的男子被诊断患有CIDP,接受大剂量静脉注射甲基强的松龙,静脉注射免疫球蛋白(IVIG),在疾病的急性期和血浆置换(PE),长期口服泼尼松,硫唑嘌呤(AZA),和霉酚酸酯(MMF)在缓解期。然而,该患者在五年内经历了六次复发,因为这些,以及对常规免疫疗法的无效反应,对RTX不宽容,选择皮下注射OFA作为预防复发的预防性治疗。在总共注射了六次OFA后,CD19+B细胞基本上被耗尽。患者已被随访超过23个月,没有复发。
该病例证明了OFA治疗复发性和难治性CIDP的有效性和良好的耐受性。需要进一步的研究来研究OFA在复发性和难治性CIDP患者中的疗效和安全性,尤其是那些对常规免疫疗法无效且对RTX不耐受的患者.
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