Progressive multifocal leukoencephalopathy in a 27-year-old lady with systemic lupus erythematosus - rheumatoid arthritis overlap syndrome
Progressive multifocal leukoencephalopathy in a 27-year-old lady with systemic lupus erythematosus - rheumatoid arthritis overlap syndrome
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DOI：
10.1016/j.jfma.2019.05.021
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发表时间：
2019-11-01
影响因子：
3.2
通讯作者：
Li, Ko-Jen
中科院分区：
文献类型：
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作者：
Cheng, Chiao-Feng;Su, Jen-Jen;Li, Ko-Jen
进行性多灶性脑白质病（PML）是一种罕见的中枢神经系统病毒性脱髓鞘疾病。免疫抑制是该病的重要危险因素。以前，PML更常见于血液恶性肿瘤和获得性免疫缺陷综合征（艾滋病）患者。在生物疾病修饰抗风湿药物时代，PML在自身免疫性疾病患者中更为常见。然而，由于其罕见性和不特异性的临床表现，在临床实践中可能难以准确诊断和立即恢复免疫，因此结果可能是悲惨的。在此，我们报告一位27岁的系统性红斑狼疮(SLE) -类风湿关节炎重叠综合征疑似累及中枢神经系统的女性。首先怀疑是神经精神性SLE。但经强化免疫抑制治疗后，病情持续恶化。因此考虑了另一种诊断。由于她的免疫抑制状态和暗含的放射学表现，怀疑PML，脑脊液中JC病毒的聚合酶链反应证实了诊断。通过减少免疫抑制剂剂量恢复免疫状态后，患者病情明显好转。我们报告这个病例是为了提高临床对罕见但可能未被诊断的疾病的警觉性。台湾医学会版权所有2019爱思唯尔台湾有限责任公司出版。
Progressive multifocal leukoencephalopathy (PML) is a rare viral demyelinating disease of central nervous system. Immunosuppression is a significant risk factor for the disease. Previously, PML developed more commonly in patients of hematological malignancy and acquired immune deficiency syndrome (AIDS). In the era of biological disease modifying anti-rheumatic drugs, PML is more frequently reported in patients of autoimmune diseases. However, for its rarity and unspecific clinical presentations, accurate diagnosis and immediate immune restoration might be difficult in clinical practice, and the outcome might thus be miserable. Herein we present a 27-year-old lady of systemic lupus erythematosus (SLE) - rheumatoid arthritis overlap syndrome with suspected involvement of central nervous system. Neuropsychiatric SLE was suspected first. However, after intensive immunosuppressive treatment, the condition kept deteriorating. An alternative diagnosis was thus considered. PML was suspected for her immunosuppressive status and suggestive radiological findings, and the diagnosis was confirmed by a polymerase chain reaction of JC virus from cerebrospinal fluid. After restoring the immune status by decreasing the dose of immunosuppressants, the condition of the patient improved significantly. We report this case to raise the importance of clinical alertness for the rare but possibly underdiagnosed disease. Copyright (C) 2019, Formosan Medical Association. Published by Elsevier Taiwan LLC.