Nived O, Bengtsson A A, Jönsen A, Sturfelt G
Department of Rheumatology, Clinical Sciences, Lund University Hospital, SE-221 85 Lund, Sweden.
Lupus. 2008 Nov;17(11):1036-41. doi: 10.1177/0961203308089445.
Progressive multifocal leukoencephalopathy (PML) is a rare, deadly demyelinating disease of the central nervous system, which is caused by a reactivation of the DNA polyomavirus JC and occurs in immunosuppressed individuals. So far, only 25 cases have been described in patients with SLE and none survived without antiviral therapy and only two cases in RA. We present four additional cases from a defined area, three in SLE, of which one survived without antiviral therapy, and one case in RA, also surviving after reduction of immunosuppressive treatment. In three of these cases, diagnosis could only be confirmed by stereotactical brain biopsy, including the two surviving cases. Thus, this article illustrates the difficulty in diagnosing progressive multifocal leukoencephalopathy, the need for brain biopsy in many cases, the importance of reduced immunosuppression as early as possible and the severe damage progressive multifocal leukoencephalopathy can cause. Furthermore, progressive multifocal leukoencephalopathy might be much more common in SLE than expected with 1 case in 800 patient-years.
进行性多灶性白质脑病（PML）是一种罕见的、致命的中枢神经系统脱髓鞘疾病，由DNA多瘤病毒JC再激活引起，发生于免疫抑制个体。迄今为止，系统性红斑狼疮（SLE）患者中仅报道过25例，未经抗病毒治疗者无一存活，类风湿关节炎（RA）患者中仅2例。我们报告了来自某特定地区的另外4例，其中3例为SLE患者，1例未经抗病毒治疗存活；1例为RA患者，在减少免疫抑制治疗后也存活。在这些病例中，有3例仅通过立体定向脑活检确诊，包括2例存活病例。因此，本文说明了进行性多灶性白质脑病的诊断困难、许多病例需要进行脑活检、尽早降低免疫抑制的重要性以及进行性多灶性白质脑病可能造成的严重损害。此外，进行性多灶性白质脑病在SLE中的实际发生率可能比预期高得多，每800患者年中有1例。
