Abstract:

ABSTRACT Objective：Neuropsychiatric (NP) involvement is severe manifestation of systemic lupus erythematosus (SLE). The purpose of this study was to investigate the clinical feature and prognosis of SLE patients with severe NP events.  Methodology：Fifty one SLE patients who had severe NP events in Jinling Hospital from 2006 to 2013 was retrospectively reviewed, satisfying the 1999 ACR criteria. Their clinical manifestations and brain magnetic resonance imaging findings were investigated. Results：They were 42 females and 9 males with an average age of 26.3±9.7 years old (range 15~64). SLE disease activity index (SLEDAI) score was 20.3±7.0 (range 8~36). A total of 101 NP events were noticed in these patients, including 92(91.1%) central nervous system events and 9(8.9%) peripheral nervous system events. The most common manifestations were seizure disorder (43.1%), followed by intractable headache (33.3%), acute confusional state (33.3%) and cerebrovascular disease (21.6%). Abnormal brain MRI features were detected in 90% of the patients, including multiple abnormal signals (38%) , cerebral infarction (34%) and white matter lesions (20%). The positive rates of anti-nuclear antibodies (ANA), anti-dsDNA antibodies, anti-C1q antibodies, anticardiolipin antibodies and lupus anticoagulant were 94.1%, 54.9%, 65.8%, 40.4% and 27.9% respectively. The vascular cell adhesion molecule (VCAM) and leukocyte adhesion test were positive in 90.9% of the patients. All patients were treated with prednisone. High dose intravenous methylprednisolone pulse therapy (0.5g for 3 days) was administered to 32 (62.7%) patients. High dose intravenous immunoglobulin (IVIG), double filtration plasmapheresis (DFPP), cyclophosphamide(CYC), low molecular heparin and anti-platelet drugs were also applied. Forty-six patients (90.2%) had improvement and were discharged (remission group), 2 (3.9%) died, 3 (5.9%) patients gave up treatment (non-remission group).The number of circulating endothelial cells (CECs) in non-remission group was significantly higher than in remission group (47.8±19.8 vs 28.5±14.4 cells/ml, p=0.014 ). Conclusion: The clinical features of SLE patient with severe NP involvement were heterogeneous, manifested by high SLE-DAI scores and significant immune and endothelial abnormalities, as well as multiple abnormal brain MRI findings. Elevation of circulating endothelial cells may be a risk factor of vicious prognosis.

Key words: Key words: Neuropsychiatric systemic lupus erythematosus    , Endothelial injury     , Circulating endothelial cells