Objectives: To investigate the prevalence, outcome and prognostic factors of neuropsychiatric systemic lupus erythematosus (NPSLE).Methods: SLE inpatients from 2005 to 2016 were included. Information on survival duration and causes of death was collected. Data were analyzed using Kaplan-Meier curves, log-rank tests and Cox proportional hazards modeling.Results: Among 1589 SLE patients, 101 (6.4%) were diagnosed with NPSLE. The overall survival rates of the NPSLE patients were 89%, 85% and 84% at 1, 3 and 5 years, respectively. The standardized mortality ratio of NPSLE patients was 11.14. The most common cause of death was NPSLE related conditions (7, 47%), including intracranial hypertension syndrome, cerebrovascular disease and motor neuron disease. The following variables were associated with death: cardiac involvement, renal involvement, diffuse NPSLE, acute confusional state, more than one NPSLE manifestation, low lymphocyte count, elevated C-reactive protein, abnormal cerebrospinal fluid (CSF) and high systemic lupus erythematosus disease activity index. Acute confusional state (p = .001), elevated intracranial pressure (p = .010) and C-reactive protein (CRP) (p = .032) were independently predictive factors of death.Conclusion: Our study demonstrates an 11.14-fold increased mortality of NPSLE patients compared with general population. NPSLE related disorders are main causes of death. Acute confusional state is the most significant predictive factor for poor prognosis.
Keywords: Neuropsychiatric systemic lupus erythematosus; causes of death; prognostic factors; standardized mortality ratio; survival.