Prevalence, outcome and prognostic factors of neuropsychiatric systemic lupus erythematosus: A real world single center study

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. © 2019, © 2019 Japan College of Rheumatology.