Physiopathologie des glomérulopathies extramembraneuses

Abstract

Membranous nephropathy (MN) is a kidney disease characterized by deposition of immune complexes and complement on the outer aspect of the glomerular capillary wall. It is responsible for a loss of serum proteins in the urine and kidney failure. During the last ten years, considerable progress has occurred in the understanding of the molecular bases of the disease with the description of three distinct mechanisms in humans. In the neonatal allo-immune form, antibodies are directed against neutral endopeptidase (NEP), a podocyte antigen absent in the mothers who become immunized against this antigen expressed by placenta cells during pregnancy. NEP was the first podocyte antigen to be identified in MN. Most adult forms of MN are autoimmune diseases without identified etiology (primary MN), linked to the production of antibodies raised against another podocyte antigen, the type-M phospholipase A2 receptor (PLA2R1). Anti-PLA2R1 antibodies are detected in 70 to 80% of patients before any immunosuppressive treatment, and only occasionally in secondary forms of MN, variants of PLAR1 and HLA-DQA1 genes are very significantly associated with occurrence of primary MN in Caucasians. The third mechanism is characterized by immunization against a foreign protein, cationic bovine serum albumin (BSA), which is involved in rare forms of MN during early childhood. This finding points to a possible role of food and environmental antigens in membranous nephropathy.

The intensity of PLA2R and C4d immunoexpression in primary membranous nephropathy

“…Membranous nephropathy (MN) is the leading cause of nephrotic syndrome in the adult population. [1][2][3][4] The disease is characterized by immune complex deposition on the outer aspect of the glomerular basement membrane. This deposition causes loss of function of the glomerular filtration barrier, which results in proteinuria.…”

“…This deposition causes loss of function of the glomerular filtration barrier, which results in proteinuria. [2][3][4] Traditionally, membranous nephropathy has been classified as primary or secondary membranous nephropathy. In 70-80% of the cases, there is no known etiology.…”