Evaluation of alternative pathway and factor B haemolytic activities in patients with systemic lupus erythematosus: correlations with the alternative pathway regulatory proteins.
The haemolytic activities of the complement alternative pathway and factor B were studied using a 51Cr-release assay in 77 sera from patients with systemic lupus erythematosus (SLE). These values were compared to those of several complement components and of the regulatory proteins beta H and C3b INA. There was a significant decrease in the alternative pathway activity, which correlated with a decrease in the haemolytic activity of factor B and with the activity of the classical pathway. There was a significant decrease in the level of one regulatory protein beta 1H, while the level of the other regulatory protein, C3b INA, was increased; however, there was a positive correlation between these two parameters. The results obtained suggest that the decreased activity of the alternative pathway observed in SLE reflects a consumption due to a triggering of the C3b amplification loop through the activation of the classical pathway. In one patient, an acquired deficiency of the capacity to activate the alternative pathway in the presence of inulin was observed.
D000328:Adult; D000936:Antigen-Antibody Complex / Q000276:immunology; D001609:Beta-Globulins / Q000032:analysis; D003167:Complement Activation; D003176:Complement C3 / Q000032:analysis; D003180:Complement C3b Inactivator Proteins / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D011415:Complement Factor B / Q000276:immunology*; D003170:Complement Pathway, Alternative; D003171:Complement Pathway, Classical; D004792:Enzyme Precursors / Q000276:immunology*; D006461:Hemolysis; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000276:immunology*; D008297:Male; D008722:Methods
D000936:Antigen-Antibody Complex; D001609:Beta-Globulins; D003176:Complement C3; D003180:Complement C3b Inactivator Proteins; D003181:Complement C4; D004792:Enzyme Precursors; D011415:Complement Factor B