Abstract
OBJECTIVE: Neuropsychiatric systemic lupus erythematosus (NPSLE) is among the most challenging manifestations of systemic lupus erythematosus (SLE), affecting the central and peripheral nervous systems with diverse symptoms. Despite its prevalence, the diagnosis and management of NPSLE remain complex due to the heterogeneity of clinical presentations and the lack of specific biomarkers. This study aims to synthesize data from case reports to better understand the clinical, biological, and imaging features of NPSLE. METHODS: A systematic review with integrated descriptive and exploratory quantitative analyses of 120 published case reports was conducted, integrating demographic, clinical, biological, and imaging data, as well as therapeutic approaches. Subgroup analyses stratified by age and sex were performed to identify descriptive patterns of presentation and outcomes. RESULTS: Age- and sex-related patterns were observed in disease presentations. Patients presenting at younger ages more frequently exhibited renal and cutaneous involvement, whereas those presenting in adulthood or at older ages more often displayed neurological and neuropsychiatric manifestations, including motor dysfunction, cognitive impairment, and mood disorders. Male patients exhibited higher rates of severe renal involvement, whereas neuropsychiatric symptoms were more frequently reported, particularly among female cases. Neuroimaging abnormalities were identified in over 80% of patients in whom imaging was performed, with common findings including white matter lesions, cortical atrophy, and focal gray matter involvement. Despite the widespread use of corticosteroids and immunosuppressants, treatment gaps persisted, particularly in the management of neuropsychiatric symptoms. CONCLUSION: This review highlights the need for the development of biomarker-driven diagnostic tools and targeted therapies to address the unmet needs of NPSLE patients, while advances in imaging and biologics hold promises for improving patient outcomes. These findings should be interpreted as hypothesis-generating, given the descriptive nature of case-report-based evidence.