RESUMO
Objective:To investigate the clinical characteristics, prognosis, and risk factors for poor prognosis of neuropsychiatric systemic lupus erythematosus (NPSLE) .Methods:Patients who were diagnosed as NPSLE between January 2009 to January 2019 in Peking University First Hospital were included. Patients with neuro-psychiatric symptoms caused by other reasons such as infection and metabolic disorders were excluded. Patients were retrospectively followed up by telephone or medical records. Continuous variables were compared by student t test or Wilcoxon rank sum test. Quantitative variables were compared by chi-square test. Survival was analyzed by Kaplan-Meier curve. Predictive factors of prognosis was estimated by using Cox regression analysis. Results:One hundred and nine NPSLE patients were included. Thirteen (11.9%) were male and 96 (88.1%) were female with a median age of 33 years old. Central nervous system involvement was predominant (89/109, 81.7%) . The most common types were headache, cerebrovascular disease and epilepsy. Cranial neuropathy was the most common type at the initial onset of systemic lupus erythematosus (SLE) , while cerebrovascular disease was more common when SLE relapsed. Patients who demonstrated NPSLE at the initiation of SLE had shorter survival time than those who got NPSLE when SLE relapsed [ (32±26) months vs (197±79) months, t=2.834, P=0.037]. Among the 105 patients with complete followed up data, the follow up time was 118.0 (1.4, 525.7) months and 53.1 (0.4, 363.0) months from the onset of SLE and NPSLE, respectively. The mortality rate was 14.3% (15/105) . The survival rates of 1-5 years were 96.2%, 94.3%, 91.0%, 89.9% and 88.3%, respectively. The survival time was (180±138) months and (33±32) months, t=3.861 , P<0.01) from the onset of SLE and NPSLE, respectively. The major causes of death were infection, NSPLE and cardiovascular disease. Cerebrovascular disease was the independent risk factor for death [ RR=3.413, 95% CI (1.049, 11.102) , P=0.041]. Conclusion:Cranial neuropathy is the most common type at the initial onset of SLE, while cerebrovascular disease is more common when SLE relapsed. Patients who had NPSLE at the initiation of SLE have shorter survival time than those who got NPSLE when SLE relapsed. Cerebrovascular disease is the independent risk factor of death of NPSLE patients.