Abstract

ObjectivesTo review the epidemiological, pathophysiological, and neurological manifestations, as well as the diagnosis and treatment of central nervous system involvement in systemic lupus erythematosus. MethodsA literature search was performed using PubMed database complemented by review of literature references listed in identified articles. There were no limits to publication date of the literature references. Only articles in English and Spanish were included. ResultsCentral nervous system involvement is one of the major causes of morbidity and mortality in systemic lupus erythematosus patients. Common disorders (cumulative incidence >5%) were cerebrovascular disease and seizures, and relatively uncommon (1-5%) were severe cognitive dysfunction, acute confusional state, psychosis, while the remaining neuropsychiatric disorders were unusual (less than 1%). Mechanisms that can lead to neuropsychiatric manifestations include intracranial vascular lesions (vasculitis and thrombosis), autoantibody production against neuronal antigens, ribosomes, and phospholipids, and inflammation related to local cytokine production. Serological tests, cerebrospinal fluid, and imaging investigations have been used to support the clinical diagnosis. Treatment is based on the use of corticosteroids, immunosuppressants, and symptomatic treatment. The use of antiplatelet and antithrombotic drugs was indicated when positive moderate-to-high titres of anti-phospholipid antibodies were present. ConclusionsThe involvement of vital organs and tissues, such as the brain, in patients with systemic lupus erythematosus, impels efforts to develop diagnostic tools for systemic lupus erythematosus associated neuropsychiatric disease to guide effective therapeutic decisions.

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