Purpose Sphingolipids, an essential signaling molecules for the biological and structural functions of cells, are increasingly recognized as playing an important signaling role in the pathophysiology of chronic inflammatory diseases. We hypothesized that the pathogenesis of systemic lupus erythematosus (SLE), a chronic autoimmune disease, is related to altered composition and dysregulation of sphingolipids.

Methods We performed liquid chromatography tandem mass spectrometry to evaluate the levels of sphingolipids in plasma from 38 women with SLE, including 11 lupus nephritis, and 30 controls. The receiver operating characteristic curve (ROC) was analyzed to calculate the area under the curves (AUC) to determine whether sphingolipids can be efficiently used to diagnose SLE. Further, Pearson’s correlation coefficient was used to analyze the correlation between sphingolipids and the disease activity markers.

Results The mean age of SLE patients was 44.5 years and the mean disease duration was 110.7 months. The levels of serum ceramide (Cer) and Cer to sphingosine-1-phosphate (S1P) ratio subspecies were increased in patients with SLE, while the levels of sphingomyelins were decreased compared to the controls. The ratio of Cer16:0 to S1P showed especially strong increments in patients with lupus nephritis, and the AUC value for discriminating lupus nephritis from controls was 0.739 (95% confidence interval, 0.581–0.898). In addition, their levels were associated with disease duration, anti-double stranded DNA antibody, SLE disease activity index 2000, and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index.

Conclusions Serum sphingolipids can be a good candidate for SLE diagnostic markers, and in particular, Cer16/S1P has the best ability to distinguish against lupus nephritis.