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APS is the association of antiphospholipid antibodies (aPL) with thromboses and/or recurrent pregnancy loss (RPL). Among patients with SLE, one-third have aPL and 10–15% have a manifestation of secondary APS. Animal studies suggested that complement activation plays an important role in the pathogenesis of thrombosis and pregnancy loss in APS. We performed a cross-sectional study on complement proteins and genes in 525 patients with aPL. Among them, 237 experienced thromboses and 293 had SLE; 111 had both SLE and thromboses, and 106 had neither SLE nor thrombosis. Complement protein levels were determined by radial immunodiffusion for C4, C3 and factor H; and by functional ELISA for mannan binding lectin (MBL). Total  C4, C4A  and  C4B  gene copy numbers (GCN) were measured by TaqMan-based realtime PCR. Two to six copies of  C4  genes are frequently present in a diploid genome, and each copy may code for an acidic C4A or a basic C4B protein. We observed significantly (a)  higher  protein levels of total C4, C4A, C4B, C3, and anticardiolipin (ACLA) IgG, (b) increased frequencies of lupus anticoagulant and males, and (c) decreased levels of complement factor H, MBL and ACLA-IgM among patients with thrombosis than those without thrombosis ( N  = 288). We also observed significantly  lower  GCNs of total  C4  and  C4A  among aPL-positive patients with both SLE and thrombosis than others. By contrast, aPL-positive subjects with SLE had significantly reduced protein levels of C3, total C4, C4A, C4B and ACLA-IgG, and higher frequency of females than those without SLE. Patients with thrombosis but  without  SLE ( N  = 126), and patients with SLE but  without  thrombosis ( N  = 182) had the greatest differences in mean protein levels of C3 ( p  = 2.6 × 10 −6 ), C4 ( p  = 2.2 × 10 −9 ) and ACLA-IgG ( p  = 1.2 × 10 −5 ). RPL occurred in 23.7% of female patients and thrombotic SLE patients had the highest frequency of RPL (41.0%;  p  = 3.8 × 10 −10 ). Compared with non-RPL females, RPL had significantly higher frequency of thrombosis and elevated C4 protein levels. Female patients with homozygous C4A deficiency  all  experienced RPL ( p  = 0.0001) but the opposite was true for patients with homozygous C4B deficiency ( p  = 0.017). These results provide new insights and biomarkers for diagnosis and management of APS and SLE.

frontiers in immunology

Opposite Profiles of Complement in Antiphospholipid Syndrome (APS) and Systemic Lupus Erythematosus (SLE) Among Patients With Antiphospholipid Antibodies (aPL)