Membranous nephropathy is well characterized histomorphologically, pathophysiologically, and clinically. Histomophological findings include immunoglobulin (IgG4) and C3 deposits that that impose a diffuse thickening of the glomerular basement membrane. In electron microscopy these deposits are electron dense. Pathophysiologically autoantibodies against podocyte antigens (PLA2R1) and the lectin pathway play a role. Clinically, the patients present with severe nephrotic syndrome.
Two forms can be distinguished the primary or idiopathic form comprises 75% of cases. The secondary form is due to diseases such as lupus erythematoides, hepatitis B, drugs, or malignant tumors.
Incidence is 1.7:100,000 with male predominance (2:1).[Error: Macro 'ref' doesn't exist]
Autoantibodies against podocyte antigens play crucial rote in pathogenesis, and polymorphisms in the PLA2R1 und HLA-DQA1 genes are strongly correlated to the development of the disease.
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OMIM.ORG article Omim 614692 |
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Orphanet article Orphanet ID 97560 |
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Wikipedia article Wikipedia EN (Membranous_glomerulonephritis) |