Membranous Nephropathy: Updates on Management

Abstract

Membranous nephropathy is a major etiology of nephrotic syndrome in adults and less frequently in children. Circulating antibodies to intrinsic podocyte antigens, such as M-type phospholipase A2 receptor, or to extrinsic proteins accumulate beneath the podocyte to cause damage via complement activation and/or other mechanisms. The availability of clinical testing for autoantibodies to M-type phospholipase A2 receptor has allowed noninvasive diagnosis of this form of membranous nephropathy and a means to monitor immunologic activity to guide immunosuppressive therapy. Treatment of membranous nephropathy includes optimal supportive care with renin-angiotensin-system blockers, lipid-lowering agents, diuretics, lifestyle changes, and additional immunosuppressive therapy in patients with an increased risk of progression to kidney failure. Rituximab has been recognized as a first-line immunosuppressive therapy for most membranous nephropathy patients with an increased risk of progressive disease, except those with life-threatening nephrotic syndrome or rapidly deteriorating kidney function from membranous nephropathy. This article discusses the major and minor antigens described in membranous nephropathy, the natural history of the disease, and guidelines for clinical management and immunosuppressive treatment.

Keywords: Autoantibodies; Membranous nephropathy; Nephrotic syndrome; PLA2R; Rituximab.