Background/Purpose: Neuropsychiatric systemic lupus erythematosus (NPSLE), especially diffuse psychiatric/neuropsychological syndromes (diffuse NPSLE), is one of the most difficult complications of the disease. For the evaluation and the diagnosis of central nervous system manifestations, including NPSLE, MRI is a very useful tool to detect the various abnormalities. We previously disclosed that patients with diffuse NPSLE and MRI abnormalities have more severe inflammation in the central nervous system compared with those without MRI abnormalities, as evidenced by poorer prognosis. Of note, acute confusional state (ACS) is the most recalcitrant manifestation in diffuse NPSLE. However, it remains unclear whether abnormalities in brain MRI still have any influences in patients with ACS. The aim of this study is to explore the influences of various factors, including brain MRI abnormalities, on the prognosis of patients with ACS.

Methods: Thirty-six patients with ACS admitted to our University Hospitals from 1992 to 2015 were exhaustively enrolled in this study. Their medical charts and brain MRI scans were reviewed. The influences of various factors on the mortality of the patients were analyzed.

Results: During the observation periods (33.7}85.4months, mean}SD), 8 of the 36 patients (22.2%) died. The causes of death were related with the disease activity of SLE, mainly NPSLE, in the 8 patients. All the fatal patients died within 8 months from the onset of ACS (3.00}2.97 months, mean}SD). Notably, all these 8 patients presented brain MRI abnormalities, whereas none of the 18 patients without MRI abnormalities died. Accordingly, the presence of brain MRI abnormalities significantly increased the mortality rate in patients with ACS.  To explore in detail the effects of various factors, including MRI abnormalities, on the mortality of patients with ACS, further analysis was carried out using Cox proportional hazards model. As shown in Table, the presence of brain MRI abnormalities, the presence of serum anti-Sm antibodies and serum IL-6 levels significantly increased the risk for mortality in multivariate analysis. 

Conclusion: These results demonstrate that patients with ACS of diffuse NPSLE with MRI abnormalities have more severe diseases, resulting in poorer prognoses. The data also indicate that serum anti-Sm antibodies and serum IL-6 are critical factors influencing the prognosis of patients with ACS. Table

Effects of various factors on the survival in patients with acute confusional state