Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Foundation, 9500 Euclid Avenue, Desk A50, Cleveland, OH 44195, USA.
St Vincent's University Hospital, Elm Park, Dublin 4, Ireland.
Nat Rev Rheumatol. 2015 Feb;11(2):119-23. doi: 10.1038/nrrheum.2014.167. Epub 2014 Oct 14.
Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic infection of the central nervous system, caused by reactivation of the ubiquitous JC virus. PML is a devastating disease that is frequently fatal, and although survival rates have improved, patients who survive PML often experience considerable neurological deficits. PML was associated with a variety of immunosuppressive therapies in the past decade, but attribution of causality is difficult owing to the presence of confounding factors and to an inadequate understanding of the underlying pathogenesis of this disease. This uncertainty has hindered efforts for shared decision-making between physicians and their patients and, in some cases, discouraged the use of potentially beneficial therapies. We propose a categorization of immunosuppressive agents according to their risk of PML to support a better-informed decision-making process when evaluating the risks and benefits of these therapies.
进行性多灶性白质脑病(PML)是一种罕见的中枢神经系统机会性感染,由普遍存在的 JC 病毒重新激活引起。PML 是一种破坏性疾病,通常是致命的,尽管生存率有所提高,但存活的 PML 患者通常会经历相当大的神经功能缺损。在过去十年中,PML 与各种免疫抑制疗法有关,但由于存在混杂因素和对这种疾病潜在发病机制的理解不足,因果关系的归因很困难。这种不确定性阻碍了医生和患者之间的共同决策,并在某些情况下阻碍了潜在有益疗法的使用。我们根据 PML 的风险对免疫抑制剂进行分类,以在评估这些疗法的风险和益处时支持更明智的决策过程。