Clinical features and mortality in Chinese with lupus nephritis and neuropsychiatric lupus: A 124-patient study

BACKGROUND: Few investigation has focused on the patients with lupus nephritis (LN) and neuropsychiatric systemic lupus erythematosus (NPSLE). This study was aimed to investigate the clinical features, mortality, and the predictors for mortality of this group of patients. MATERIALS AND METHODS: Medical records were retrospectively reviewed in Sun Yat-sen Memorial Hospital from 1996 to 2012. Data of demographic information, clinical manifestations, laboratory tests, SLE disease activity index 2000 (SLEDAI-2K) score, diagnosis, complications, treatment, and mortality was collected. RESULTS: A total of 124 patients were included in our study. Thirty-five (29.1%) patients had glomerular filtration rate <60 ml/min/1.73 m(2), while 24 (19.4%) experienced acute kidney injury (AKI). Thirteen of the 19 American College of Rheumatology defined NPSLE syndromes were identified. The most frequent manifestation was seizure disorder (56/124, 45.2%), followed by psychosis (37/124, 29.8%) and cerebrovascular disease (35/124, 28.2%). One hundred and five (84.7%) patients had SLEDAI-2K scores ≥15, the mean of which was 21.5 ± 6.2. The mortality during hospitalization was 12.9% (16/124) with NP involvement itself being the leading cause of death (7/16, 43.8%). Multivariate logistic regression confirmed that age <14 years at onset of NPSLE (odds ratios [OR]: 9.95, 95% confidence intervals [CI]: 1.43-69.36, P = 0.020), AKI (OR: 10.40, 95% CI: 2.33-46.48, P = 0.002) and pneumonia (OR: 4.52, 95% CI: 1.14-17.96, P = 0.032) were risk factors for mortality, while cyclophosphamide (CYC) treatment (OR: 0.09, 95% CI: 0.02-0.54, P = 0.008) was a protective factor. CONCLUSION: Most of SLE patients with LN and new-onset NPSLE are in an active disease state. NP manifestation itself was the leading cause of death during hospitalization. Childhood-onset NPSLE, AKI and pneumonia might be predictors of mortality, whereas CYC treatment might improve the prognosis.