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  • Review Article
  • Published:

Diagnosis and management of neuropsychiatric SLE

Nature Reviews Rheumatology volume 10, pages 338–347 (2014)Cite this article

Subjects

Key Points

  • Neuropsychiatric events are common in patients with systemic lupus erythematosus (SLE) but only one-third of such events are attributed directly to SLE

  • Autoimmune-mediated inflammatory injury and vascular injury are separate pathogenetic mechanisms responsible for neuropsychiatric SLE (NPSLE)

  • The diagnosis of NPSLE is determined primarily by clinical assessment

  • Investigations in support of the clinical diagnosis include measurement of autoantibodies, analysis of cerebrospinal fluid, electrophysiological studies, neuropsychological assessment and neuroimaging

  • Treatment options for NPSLE include addressing comorbidities, symptomatic therapies, immunosuppression and anticoagulation

Abstract

Nervous system involvement in systemic lupus erythematosus (SLE) can manifest as a range of neurological and psychiatric features, which are classified using the ACR case definitions for 19 neuropsychiatric syndromes. Approximately one-third of all neuropsychiatric syndromes in patients with SLE are primary manifestations of SLE-related autoimmunity, with seizure disorders, cerebrovascular disease, acute confusional state and neuropathy being the most common. Such primary neuropsychiatric SLE (NPSLE) events are a consequence either of microvasculopathy and thrombosis, or of autoantibodies and inflammatory mediators. Diagnosis of NPSLE requires the exclusion of other causes, and clinical assessment directs the selection of appropriate investigations. These investigations include measurement of autoantibodies, analysis of cerebrospinal fluid, electrophysiological studies, neuropsychological assessment and neuroimaging to evaluate brain structure and function. Treatment involves the management of comorbidities contributing to the neuropsychiatric event, use of symptomatic therapies, and more specific interventions with either anticoagulation or immunosuppressive agents, depending upon the primary immunopathogenetic mechanism. Although the prognosis is variable, studies suggest a more favourable outcome for primary NPSLE manifestations compared with neuropsychiatric events attributable to non-SLE causes.

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Figure 1: Factors contributing to neuropsychiatric events in patients with SLE.
Figure 2: Negative effect of NP events on HRQoL in patients with SLE.22
Figure 3: Autoimmune pathogenesis of NPSLE.
Figure 4: Physician-generated outcome scores for NP events at enrolment into an international inception cohort of patients with SLE.23

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Acknowledgements

The author's work is supported by the Government of Canada, Canadian Institutes of Health Research grant number MOP 86526.

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  1. Division of Rheumatology, Department of Medicine and Department of Pathology, Capital Health and Dalhousie University, Halifax, B3H 4K4, NS, Canada

    John G. Hanly

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Hanly, J. Diagnosis and management of neuropsychiatric SLE. Nat Rev Rheumatol 10, 338–347 (2014). https://doi.org/10.1038/nrrheum.2014.15

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