Abstract
Membranous nephropathy (MN) is a non-inflammatory organ-specific autoimmune disease which affects the kidney glomerulus, resulting in the formation of immune deposits on the outer aspect of the glomerular basement membrane, complement-mediated proteinuria, and severe renal failure in 30 % of patients. In the last 10 years, substantial advances have been made in the understanding of the molecular bases of MN, with the identification of several antigens and predisposing genes in children and adults. These ground-breaking findings already have a major impact on diagnosis and monitoring and to some extent on therapies. However, there is evidence that the disease is more complex and involves a variety of antigen–antibody systems and genes involved in immune response, progression, recovery, and protective mechanisms. We herein review these recent findings which open new perspectives of research. Understanding the complex pathogenesis of MN will offer many opportunities for future therapeutic interventions and will hopefully have a major impact on patient care. New insights into the molecular mechanisms of MN may also enlighten the pathogenesis of organ-specific autoimmune diseases.
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Acknowledgments
Research is supported by European Research Council Grant ERC-2012-ADG_20120314 (Grant Agreement 322947), Agence Nationale pour la Recherche Programme Blanc SVSE1 (2012) Decision ANR-12-BSE1-0002-01, Fondation pour la Recherche Médicale Equipe FRM 2012 grant, and 7th Framework Programme of the European Community Contract 2012-305608 (European Consortium for High-Throughput Research in Rare Kidney Diseases).
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This article is a contribution to the special issue on Immunopathology of Glomerular Diseases - Guest Editors: P. Ronco and J. Floege
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Debiec, H., Ronco, P. Immunopathogenesis of membranous nephropathy: an update. Semin Immunopathol 36, 381–397 (2014). https://doi.org/10.1007/s00281-014-0423-y
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DOI: https://doi.org/10.1007/s00281-014-0423-y