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Rheumatology Advance Access originally published online on December 15, 2007
Rheumatology 2008 47(3):256-262; doi:10.1093/rheumatology/kem319
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© The Author 2007. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

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Neuropsychiatric lupus and reversible posterior leucoencephalopathy syndrome: a challenging clinical dilemma

A. Mak1, B. P. L. Chan2, I. B. Yeh3, R. C. M. Ho4, M. L. Boey1, P. H. Feng1, D. R. Koh1 and B. K. C. Ong2

1Division of Rheumatology, 2Division of Neurology, Department of Medicine, 3Department of Diagnostic Imaging and 4Department of Psychological Medicine, National University Hospital, National University of Singapore, Singapore.

Correspondence to: A. Mak, Assistant Professor of Medicine, Division of Rheumatology, Department of Medicine, National University Hospital, National University of Singapore, 5 Lower Kent Ridge Road, Singapore 119074. E-mail: mdcam{at}nus.edu.sg


  Abstract

Reversible posterior leucoencephalopathy syndrome (RPLS) has been increasingly recognized and reported in the literature. While the condition has been well described in patients with acute hypertension, pre-eclampsia, eclampsia, post-transplantation and chemotherapy, RPLS has been increasingly identified in patients with autoimmune diseases such as systemic lupus erythematosus (SLE). Though experience in the diagnosis and management of RPLS in patients with SLE is likely accumulating, few have systematically worked out the strategy to distinguish RPLS from neuropsychiatric SLE (NPSLE) and lupus-related complications of the central nervous system (CNS). Prompt recognition of, and differentiation between, these conditions is essential since their clinical presentations substantially overlap and yet their management strategy and subsequent outcomes can be entirely different. Indeed, inappropriate treatment such as augmentation of immunosuppression may be detrimental to patients with RPLS. A high index of suspicion of RPLS, prompt magnetic resonance imaging of the brain, including diffusion imaging, exclusion of CNS infection and metabolic derangement, a comprehensive medication review accompanied by timely and aggressive control of blood pressure and seizure are keys to successful management of RPLS. Such treatment strategy ensures a very high chance of total neurological recovery in lupus patients with RPLS.

KEY WORDS: Reversible posterior leucoencephalopathy syndrome, Systemic lupus erythematosus, Neurological, Differentiation, Treatment strategy

Submitted 10 August 2007; revised version accepted 31 October 2007.
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