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Neuro-psychiatric manifestations in systemic lupus erythematosus: new concepts from a mono-center retrospective study

  • Riehl, Diane
Publication Date
Sep 12, 2018
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Background: In 1999, the American College of Rheumatology (ACR) defined 19 syndromes of neuro-psychiatric manifestations occurring during systemic lupus erythematosus (NPSLE). The aim of this study was to describe the clinical, immunological, imaging presentations, treatments and outcomes of NPSLE 19 years later.Patients and methods: We conducted a retrospective study between 2005 and 2018. Patients were classified in 4 groups: neuro-psychiatric (NP) manifestations related to antiphospholipid antibodies (APL), focal NP manifestations, diffuse NP manifestations and combined diffuse and focal NP manifestations.Results: 81 patients (70 women, median age at SLE diagnosis 27 years [range: 12-68]) were included: 28 patients (34.6%) with NP manifestations related to APL, 8 patients (9.9%) with focal manifestations, 30 patients (37%) with diffuse manifestations and 15 (18.5%) with combined manifestations. Disease activity evaluated with Systemic Lupus Erythematosus Activity Index 2000 (SLEDAI-2K) was significantly different between groups (p=0.0001). The number of neurological relapses and neurological sequelae were significantly different between groups (p=0.038 and p=0.01 respectively): in the focal group, 3/7 patients (43%) had neurological relapses and 5/6 (83%) had neurological sequelae. Corticosteroids and hydroxychloroquine were prescribed in 91% and 95% of patients respectively, and cyclophosphamide in 59%.Conclusion: This classification of NPSLE in 4 groups based on assumed pathogenic mechanisms enables to separate patients with different clinical presentations and different outcomes. It could help to guide therapeutic care.

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