Background and aims To investigate the survival rate and prognostic factors of neuropsychiatric systemic lupus erythematosus (NPSLE) in a cohort.

Methods A total of 101 NPSLE inpatients diagnosed in a single centre from 2005 to 2016 were included. Information on survival status, date and causes of death was acquired by follow-up. Data were analysed using Kaplan–Meier curves, log-rank tests and Cox proportional hazards modelling.

Results The overall survival rates of the NPSLE cohort were 89%, 85% and 84% at 1, 3 and 5 years respectively. The standardised mortality ratio (SMR) of NPSLE patients was 11.14. The most common cause of death was NPSLE (7, 47%), including intracranial hypertension syndrome (4, 27%), cerebrovascular disease (2, 13%) and motor neuron disease (1, 7%). The remaining causes included other SLE complications (3, 20%) and infection (2, 13%). Crude analysis showed that the following variables at diagnosis were associated with a shorter survival period: cardiac involvement, renal involvement, diffuse NPSLE, acute confusional state, number of NPSLE manifestations >1, lymphocyte <10⁹/L, elevated C-reactive protein(CRP), abnormal cerebrospinal fluid and high systemic lupus erythematosus disease activity index (SLEDAI). On multivariate analysis, acute confusional state (p=0.004), high SLEDAI (p=0.020) and elevated CRP (p=0.023) were independently prognostic of death.

Conclusions Our study demonstrate an 11.14-fold increased mortality of NPSLE patients compared with general population. NPSLE itself is the most frequent cause of death. Acute confusional state is the most significant predictive factor for poor prognosis.