FH and nephrotic syndrome: Patients with properdin-dependent activity (sCSA−/sPCSA+ or FH-CSA−/FH-PCSA+) were more likely to have subendothelial deposits in kidney biopsy, and to develop nephrotic syndrome during disease course, while intramembranous highly electron dense deposits were more prevalent in patients with properdin-independent activity (sCSA+/sPCSA+ or FH-CSA+/FH-PCSA+) (Figure 8, Table 5 and Supplementary Table 1).