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Membranous nephropathy 

Membranous nephropathy
Membranous nephropathy

An S. De Vriese

, and Fernando C. Fervenza

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date: 02 March 2021

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in Caucasians adults. It may also present with asymptomatic proteinuria. Its defining feature is the presence of subepithelial immune deposits, localized between the podocyte and the glomerular basement membrane. Aetiology—primary MN (80% of cases) is caused in most cases by antibodies against the M-type phospholipase A2 receptor (PLA2R). Secondary MN occurs as a consequence of drugs, malignancy, or autoimmune disease. Prognosis—the clinical course of primary MN is variable: spontaneous complete remission of proteinuria occurs in 20 to 30% and progressive kidney failure develops in 20 to 40% over 5 to 15 years. Patients with gross proteinuria (>8 g/day) are at high risk of progression, as are those with a high and rising anti-PLA2R antibody level. Management—patients at low risk of progression have an excellent long-term prognosis and should be treated conservatively without immunosuppression. Patients at medium and high risk for progression benefit from immunosuppression in addition to conservative treatment. Corticosteroid monotherapy is ineffective in primary MN and should not be used. Standard treatment regimens include corticosteroids with alkylating agents (chlorambucil, cyclophosphamide), corticosteroids with mycophenolate mofetil, and calcineurin inhibitors (ciclosporin, tacrolimus). Early experience with rituximab has given some promising results.

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