D'Amico Emanuele, Zanghì Aurora, Leone Carmela, Tumani Hayrettin, Patti Francesco
Multiple Sclerosis Center, Policlinico G. Rodolico, Via Santa Sofia, 78, Catania, 95123, Italy.
Klinik und Poliklinik für Neurologie der Universität Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany.
Drug Saf. 2016 Dec;39(12):1163-1174. doi: 10.1007/s40264-016-0461-6.
Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection of the central nervous system caused by the John Cunningham virus (JCV) that has been associated with therapeutic immunosuppression in patients with multiple sclerosis (MS). So far, more than 600 cases of PML have been reported in association with natalizumab administration. There have also been confirmed cases of PML in individuals who received fingolimod and dimethyl fumarate without previous natalizumab treatment. The new licensed disease-modifying therapies for MS carry the risk of immunosuppressant and so of JCV reactivation. Various factors have been identified with increased risk of developing PML, including a positive JCV serology, natalizumab administration for >2 years, and prior use of immunosuppressive agents. Clinicians can employ such tools for patients' risk stratification, but the incidence of PML among patients receiving natalizumab therapy has not changed. In this review we outline the current state of understanding of PML pathogenesis and patients' risk stratification. The landscape of MS is dramatically changing and knowledge of the side effects of the licensed therapies is imperative to enable optimal decision making.
进行性多灶性白质脑病(PML)是一种由约翰·坎宁安病毒(JCV)引起的罕见的中枢神经系统机会性感染,与多发性硬化症(MS)患者的治疗性免疫抑制有关。到目前为止,已有600多例与那他珠单抗治疗相关的PML病例报告。在未接受过那他珠单抗治疗而接受芬戈莫德和富马酸二甲酯治疗的个体中也确诊了PML病例。MS的新型获批疾病修正疗法存在免疫抑制风险,因此存在JCV重新激活的风险。已确定多种因素会增加发生PML的风险,包括JCV血清学阳性、使用那他珠单抗治疗超过2年以及既往使用免疫抑制剂。临床医生可利用这些工具对患者进行风险分层,但接受那他珠单抗治疗的患者中PML的发病率并未改变。在本综述中,我们概述了目前对PML发病机制和患者风险分层的理解情况。MS的情况正在发生巨大变化,了解获批疗法的副作用对于做出最佳决策至关重要。