Objective: To analyze the clinical significance of anti- ubiquitin C-terminal 
hydrolase L1(UCHL-1)autoantibodies in neuropsychiatric systemic lupus 
erythematosus (NPSLE). Methods: Autoantibodies in cerebrospinal fluid specimen 
of 56 inpatients were detected by using indirect enzyme-linked immunosorbent 
assay (ELISA) and the fullmedical history and clinical manifestations were 
analyzed retrospectively. Results: The levels of anti-UCHL-1 autoantibodies in 
NPSLE group were significant higher than that in other controls (P<0.05). The 
positive rate of anti-UCHL-1 autoantibodies in NPSLE group was 23.7% (9/38), 
which was higher than that in the control groups (0%). A significant difference 
of anti-UCHL-1 autoantibodies was observed in the patients with blood system 
involvement (P=0.012). The positive rates of anti-UCHL autoantibodies in the 
patients with negative SLE related autoantibodies including AnuA, anti-dsDNA, 
Acl, anti-nRNP, anti-rRNP and anti-Smantibody negative were 41.7%, 29.4%, 29.2%, 
25.9%, 25.0%, 25.0%, respectively.The levels of anti-UCHL-1 autoantibodies had a 
positive correlation with 24-hours proteinuria (r=0.361, P=0.039). Conclusion: 
Anti-UCHL-1 autoantibodies had promising value in the diagnosis of 
neuropsychiatric systemic lupus erythematosus.