Membranous nephropathy is an autoimmune disease caused in most cases by binding of circulating antibodies to antigens on podocytes. PLA2R1 and THSD7A have been identified as target antigens in 70-80 % and 2-3 % of patients, respectively. The detection of PLA2R1- and THSD7A-antibodies in the blood and staining of renal biopsies for the respective antigens allow the correct diagnosis of PLA2R1- and THSD7A-associated membranous nephropathy, respectively, in practically 100 % of cases. Measurement of PLA2R1- and THSD7A-antibodies is helpful in order to further individualize the decision whether to perform a renal biopsy in patients with suspected membranous nephropathy. PLA2R1-antibody level is a strong predictor for remission of proteinuria, loss of renal function and relapse of disease. Moreover, treatment decisions in patients with PLA2R1-associated MN are increasingly based on the PLA2R1-antibody levels. Rituximab was shown to be non-inferior to ciclosporine A to induce remission of proteinuria after 12 months. After 24 months rituximab was superior to ciclosporine A for the same endpoint. Development of novel treatment strategies, focusing on disease pathogenesis, remains highly relevant for these patients.
