WCN26-4813 SUCCESSFUL TREATMENT OF IMMUNOSUPPRESSIVE THERAPY AND LDL-APHERESIS FOR NEPHROTIC SYNDROME CAUSED BY FIBRONECTIN GLOMERULOPATHY DIAGNOSED BY IMMUNOSTAINING AND GENETIC ANALYSIS

Fibronectin glomerulopathy (FNG) is an autosomal dominant hereditary kidney disease caused by pathogenic variants in the FN1 gene, which encodes plasma fibronectin. Mutant fibronectin abnormally deposits within the glomerular mesangium and subendothelial regions, leading to progressive glomerular injury. FNG is typically diagnosed in young adulthood. Most patients present with asymptomatic proteinuria or chronic glomerulonephritis, while nephrotic syndrome occurs in approximately 30% of reported cases.