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Neuropsychiatric symptoms affect up to half of the patients with SLE. The effect on disease severity, quality of life, and prognosis is extremely heterogeneous. Symptoms of neuropsychiatric SLE (NPSLE) range from mild diffuse conditions to acute life-threatening events. Although the underlying mechanisms are still largely unraveled, several pathogenic pathways have been identified, such as antibody-mediated neurotoxicity, vasculopathy due aPL, and cytokine-induced neurotoxicity. The chapter focuses on the central nervous system manifestations of NPSLE, with special emphasis on classification, diagnostic, and treatment aspects.

Original publication





Book title

Rare Diseases of the Immune System

Publication Date



113 - 131