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Logo of annrheumdAnnals of the Rheumatic DiseasesVisit this articleSubmit a manuscriptReceive email alertsContact usBMJ
Ann Rheum Dis. 2003 January; 62(1): 50–57.
PMCID: PMC1754279

Infectious CNS disease as a differential diagnosis in systemic rheumatic diseases: three case reports and a review of the literature


Background: 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.

Objective: To differentiate between CNS involvement and CNS infection in systemic rheumatic diseases.

Methods and results: 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).

Discussion: 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.

Figure 1
(A) Coronal MRI scan of the brain showing confluent foci (arrows) of hyperintensity in the deep and subcortical white matter and subdural space on inversion recovery sequences (patient 1). (B) Electron micrograph of a brain biopsy specimen (patient 1): ...
Figure 2
(A) Numerous ring-enhancing cortical lesions (arrows) with perifocal oedema are demonstrated on the gadolinium-enhanced coronal T1 weighted MR image. (B) The diffusion weighted axial MR image demonstrates a high signal intensity of the lesions (arrows). ...
Figure 3
(A) Axial inversion recovery sequence MRI scan of the brain demonstrates numerous confluent hyperintense lesions in the deep as well as subcortical white matter (arrows) with discrete involvement of the cortex, compatible with vasculitis or viral infection. ...
Figure 4
Diagnostic approach to brain diseases in immunosuppressed patients with pre-existing rheumatic diseases. The measurement of IgG, complement level, and CD4/8+ T cell count is not evidence based.

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