Summary
Idiopathic membranous nephropathy (IMN) is an autoimmune disease without specific aetiology and defined by proteinuria that is often in the nephrotic range and granular deposits of IgG among the glomeruli. It is the causative renal disease in about 3% of patients requiring dialysis. About 30% of patients have an unfavourable outcome and develop terminal renal failure, generally after 10 years. Massive proteinuria is the only reliable clinical predictor of the risk of renal failure.
The incidence of thrombotic events, especially renal vein thrombosis, is high in IMN, and preventive anticoagulation with anti-vitamin K agents, subcutaneous heparin or low-molecular-weight heparin may thus be of benefit. Immunological treatment remains to be of proven benefit for the overall population of patients with IMN, but may benefit patients with a rapid decline in renal function and those with predictors of poor outcome (mainly massive proteinuria). Although retrospective studies support the first-line use of corticosteroids, controlled trials have not proven their efficacy. Alkylating agents (chlorambucil and cyclophosphamide), alone or with corticosteroids, may be effective; the ‘Ponticelli’ regimen of corticosteroids plus chlorambucil is potentially the reference treatment for IMN. Intravenous immunoglobulins may be an alternative in older patients. Cyclosporin given for at least 1 year has recently been proposed for severely nephrotic patients unresponsive to corticosteroids and alkylating agents. In patients with no predictors of poor outcome, a reduction in proteinuria may be an important therapeutic goal and can be achieved with ACE inhibitors.
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Rostoker, G., Weil, B. Therapy of Idiopathic Membranous Nephropathy. Clin. Immunother. 6, 7–27 (1996). https://doi.org/10.1007/BF03259349
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DOI: https://doi.org/10.1007/BF03259349