Abstract
Although aberrant complement activation is involved in the pathogenesis of systemic lupus erythematosus (SLE), the role of complement regulatory proteins in disease activity of SLE remains limited. We enrolled the pediatric-onset SLE patients from our cohort study over 10 years. The clinical and laboratory data including SLEDAI disease activity score, and serum complement factor H (CFH), CFI, CD46, C5a, and C5b-9 in the active and remission phases were determined. Glomerular C5b-9 deposition as a complement activity marker was also examined. Forty patients (35 female and 5 male, aged 13.9 ± 3.8 years) met the criteria of investigation were assessed. Fever and kidney were the most common symptom and organ involved, respectively. Mean SLEDAI in the active and remission phases were 12.6 vs 1.7, respectively. All patients exhibited lower serum C3, C4, CFH and CFI and higher serum anti-dsDNA and CD46 in the active pahse. There was a significant difference in serum CFH, CFI and CD46 between active and remissive phases. Serum CFI but not CFH and CD46 level was negatively correlated with SLEDAI score in active phase. Compared to classical activity markers, serum CFI was superior to C4 and anti-dsDNA in reflecting disease activity and also significantly correlated with white blood count and hemoglobin. Glomerular C5b-9 depositions were detected in patients with nephritis during active phase but not in disease controls. Serum CFI level may not only be a promising biomarker for disease activity of SLE, but also reflects the hematological features of SLE.
Highlights
The aberrant complement system resulting in loss of self-tolerance, is one of the abnormalities of the immune system involved in the etiopathogenesis of systemic lupus erythematosus (SLE)
To the best of our knowledge, this is the first study demonstrating the correlation between serum Complement Factor I (CFI) levels and disease activity of SLE
We found that there were significant changes in the serum levels of complement factor H (CFH), CFI and Cluster of Differentiation 46 (CD46) between the active and remission phase, but only serum CFI levels were statistically correlated with Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)
Summary
The aberrant complement system resulting in loss of self-tolerance, is one of the abnormalities of the immune system involved in the etiopathogenesis of systemic lupus erythematosus (SLE). The excessive generation of end complement products caused by complement activation at the site of immune complex deposition leads to tissue inflammation and injury [3, 4]. Complement activation is a proteolytic cascade tightly controlled by complement regulatory proteins (CRPs) including cell membrane-bound (CD46) and soluble proteins (Complement factor H/CFH and www.impactjournals.com/oncotarget complement factor I/CFI). Deficiency or defects in CFH and CFI are associated with complement-mediated hemolytic uremic syndrome, hemolysis, and thrombosis. The activity of soluble and membrane-bound CRPs responsible for modulating the severity of disease in patients with SLE remains unexplored
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