Maillart Elisabeth, Dubessy Anne-Laure, Shor Natalia, Piljan Mathilde, Decombe Rene, Lubetzki Catherine, Stankoff Bruno, Marignier Romain, Beigneux Ysoline
APHP, Pitié-Salpêtrière Hospital, Department of Neurology, and Centre de Référence des Maladies Inflammatoires Rares du Cerveau et de la Moelle, Paris, France.
APHP, Pitié-Salpêtrière Hospital, Neuroradiology Department, Paris, France.
Neurology. 2025 Mar 11;104(5):e213399. doi: 10.1212/WNL.0000000000213399. Epub 2025 Feb 6.
Several treatments have been approved for the prevention of attacks in AQP4-IgG+ neuromyelitis optica spectrum disorder (NMOSD). However, because of the rarity of the disease, little is known concerning how to switch from one treatment to another in case of lack of effectiveness or side effects. In this article, we report a severe attack in a patient with NMOSD after switching from eculizumab to satralizumab.
A 44-year-old woman with NMOSD was treated with azathioprine and then rituximab, without optimal control of the disease. Eculizumab was initiated with clinical efficacy. Two years later, after the onset of rheumatoid arthritis and because of difficult venous access, a switch to satralizumab was proposed.
After switching, a severe attack occurred 11 weeks after the last eculizumab infusion. Severe tetraplegia was related to a new extensive cervical lesion associated with a tumefactive lesion of the corpus callosum. The patient was treated with 10 infusions of methylprednisolone and 10 plasma exchanges. Eculizumab was reintroduced 20 days after symptom onset. Three months later, mild improvement was observed.
In clinical practice, in case of intolerance or side effects, anticomplement therapy should be switched to another NMOSD treatment with caution because of a high risk of relapse.
This case report provides Class IV evidence that eculizumab should be stopped with caution and switched to another treatment immediately. This is a single observational study without controls.
几种治疗方法已被批准用于预防水通道蛋白4-IgG阳性视神经脊髓炎谱系障碍（NMOSD）的发作。然而，由于该疾病罕见，对于在治疗无效或出现副作用时如何从一种治疗方法转换到另一种治疗方法知之甚少。在本文中，我们报告了一名NMOSD患者在从依库珠单抗转换为萨特利珠单抗后发生的一次严重发作。
一名44岁的NMOSD女性患者先接受硫唑嘌呤治疗，后接受利妥昔单抗治疗，但疾病控制不佳。开始使用依库珠单抗后临床疗效良好。两年后，在类风湿性关节炎发作且静脉通路困难后，建议转换为萨特利珠单抗。
转换治疗后，在最后一次输注依库珠单抗11周后发生了一次严重发作。严重四肢瘫与新出现的广泛颈椎病变以及胼胝体肿胀性病变有关。该患者接受了10次甲泼尼龙输注和10次血浆置换治疗。症状出现20天后重新使用依库珠单抗。三个月后，观察到轻度改善。
在临床实践中，在出现不耐受或副作用的情况下，由于复发风险高，抗补体治疗应谨慎转换为另一种NMOSD治疗方法。
本病例报告提供了IV级证据，即应谨慎停用依库珠单抗并立即转换为另一种治疗方法。这是一项无对照的单观察性研究。
