Abstract
Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in white adults. It is defined by the presence of subepithelial immune deposits localized between the podocyte and the glomerular basement membrane (GBM) on electron microscopy examination. Primary MN is a kidney-limited autoimmune disease and accounts for about 80% of cases. The discovery of four major podocytes antigens, the M-type phospholipase A2-receptor 1 (PLA2R), the thrombospondin type 1 domain containing 7a (THSD7A) protein, the neural epidermal growth factor-like 1 protein (NELL-1), and semaphorin 3B, has revolutionized our understanding of the pathogenesis of MN. About 20% of MN cases are categorized as secondary, because they occur in the context of active malignancy, systemic autoimmune disease, paraproteinemia, infection, or medication use. Exostosin 1/exostosin 2 (EXT1/EXT2) are recently discovered target antigens in patients with MN and systemic autoimmune disease. The clinical course of primary MN is variable: Up to 30% of patients undergo spontaneous remission, but more than 40% of untreated patients with nephrotic syndrome eventually develop kidney failure. Mounting evidence suggests that anti-PLA2R antibody levels help in assessing prognosis and response to treatment. Genetic studies are elucidating predisposing factors for development of the disease. General treatment measures for MN include blood pressure control, minimization of proteinuria with renin-angiotensin system inhibition, treatment of dyslipidemia, and, in select patients, anticoagulation. Immunosuppressive therapy should be reserved for those who are at highest risk for developing progressive kidney failure and includes glucocorticoids combined with a cytotoxic agent (preferably cyclophosphamide), rituximab, or calcineurin inhibitors.
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