Immunopathogenesis of membranous nephropathy: an update.
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.
Sorbonne Universités, UPMC Univ Paris 06, UMR_S 1155, 75005, Paris, France, hanna.debiec@upmc.fr.
Hanna
H
Debiec
Pierre
P
Ronco
D000328:Adult; D002648:Child; D002675:Child, Preschool; D003165:Complement System Proteins / Q000276:immunology*; D050533:Glomerular Basement Membrane / Q000276:immunology / Q000473:pathology; D015433:Glomerulonephritis, Membranous / Q000276:immunology / Q000473:pathology / Q000628:therapy; D006801:Humans; D050199:Podocytes / Q000276:immunology / Q000473:pathology; D011507:Proteinuria / Q000276:immunology / Q000473:pathology / Q000628:therapy
D003165:Complement System Proteins