Warnatz K, Peter H H, Schumacher M, Wiese L, Prasse A, Petschner F, Vaith P, Volk B, Weiner S M
Department of Rheumatology and Clinical Immunology, Medizinische Klinik, University Hospital, Freiburg, Hugstetter Strasse 55, 79106 Freiburg, Germany.
Ann Rheum Dis. 2003 Jan;62(1):50-7. doi: 10.1136/ard.62.1.50.
Immunosuppressive treatment of rheumatic diseases may be associated with several opportunistic infections of the brain. The differentiation between primary central nervous system (CNS) involvement and CNS infection may be difficult, leading to delayed diagnosis.
To differentiate between CNS involvement and CNS infection in systemic rheumatic diseases.
Three patients with either longstanding or suspected systemic rheumatic diseases (systemic lupus erythematodes, Wegener's granulomatosis, and cerebral vasculitis) who presented with various neuropsychiatric symptoms are described. All three patients were pretreated with different immunosuppressive drugs (leflunomide, methotrexate, cyclophosphamide) in combination with corticosteroids. Magnetic resonance imaging of the brain was suggestive of infectious disease, which was confirmed by cerebrospinal fluid analysis or stereotactic brain biopsy (progressive multifocal leucoencephalopathy (PML) in two and nocardiosis in one patient).
More than 20 cases of PML or cerebral nocardiosis in patients receiving corticosteroids and cytotoxic drugs for rheumatic disease have been reported. The clinical aspects of opportunistic CNS infections and the role of brain imaging, cerebrospinal fluid analysis and stereotactic brain biopsy in the differential diagnosis are reviewed.
风湿性疾病的免疫抑制治疗可能与多种脑部机会性感染相关。原发性中枢神经系统(CNS)受累与CNS感染之间的鉴别可能困难,导致诊断延迟。
鉴别系统性风湿性疾病中的CNS受累与CNS感染。
描述了3例患有长期或疑似系统性风湿性疾病(系统性红斑狼疮、韦格纳肉芽肿病和脑血管炎)且出现各种神经精神症状的患者。所有3例患者均预先接受了不同的免疫抑制药物(来氟米特、甲氨蝶呤、环磷酰胺)与皮质类固醇联合治疗。脑部磁共振成像提示为感染性疾病,经脑脊液分析或立体定向脑活检得以证实(2例为进行性多灶性白质脑病(PML),1例为诺卡菌病)。
已有超过20例接受皮质类固醇和细胞毒性药物治疗风湿性疾病的患者发生PML或脑部诺卡菌病的报道。对机会性CNS感染的临床特征以及脑成像、脑脊液分析和立体定向脑活检在鉴别诊断中的作用进行了综述。