About this Research Topic
Neuropsychiatric systemic lupus erythematosus represents one of the most challenging aspects of this multisystem disease, illustrated in the wide heterogeneity of different manifestations described in the original 1999 ACR nomenclature. Its pathogenesis is thought to involve autoantibody- mediated neuronal or vascular injury, intrathecal production of inflammatory cytokines, disruption of the blood – brain barrier (BBB), and accelerated atherosclerosis, at variable extent.
The challenge with NPSLE lies in the fact that specific markers to aid in proper diagnosis or response to therapy are persistently lacking. The relatively high frequency of certain neuropsychiatric (NP) manifestations in the general population makes the attribution of a NP manifestation (ie. “primary vs. “secondary” NPSLE) to SLE demanding. Nevertheless, the latter years have witnessed significant progress in this process of attribution, with respective models being tested in clinical practice.
Lack of specificity for NPSLE is also illustrated in the often-normal conventional brain imaging of patients with established NPSLE. In recent years, novel neuroimaging modalities (functional MRI, diffusion-weighted imaging, FDG-PET) may enable the detection of subtle structural and metabolic aberrations in brain regions of NPSLE patients. Finally, guidance for treatment of NPSLE is hampered by the paucity of randomized controlled trials and current therapeutic approaches remain at large empirical. Based on the presumed underlying mechanism, inflammatory or thrombotic, the balance for choice of therapy is skewed towards anti-inflammatory or antithrombotic therapy, respectively.
Keywords: SLE, central nervous system, pathogenesis, attribution, therapy
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