Membranoproliferative glomerulonephritis -animated quick review

Author: Rewise MD

Membranoproliferative glomerulonephritis (MPGN) is a type of nephritic-nephrotic kidney disorder characterized by alterations in the glomerular basement membrane, proliferation of glomerular cells, and leukocyte infiltration. MPGN accounts for approximately 10 to 20% of cases of nephrotic syndrome in both children and young adults and is divided into two major categories on the basis of distinct immunofluorescent and pathologic differences called type I and type II MPGN. In type I MPGN, there is evidence of immune complex deposition in the glomerulus with activation of both the classical and alternative complement pathways. In many cases, the antigens involved in MPGN are thought to be derived from infectious agents such as hepatitis C and hepatitis B viruses. By light microscopy, both types of MPGN demonstrate enlarged hypercellular glomerular caused by proliferation of cells in the mesangium.

Electron microscopy shows a glomerular basement membrane that is thickened and the glomerular capillary wall often shows a tram-track appearance caused by duplication, commonly called splitting, of the basement membrane by ingrowth of the mesangium. Specifically, type I MPGN is characterized by the presence of discrete subendothelial immune complexes. Immunofluorescence demonstrates a granular pattern of C3 deposition. Type II MPGN, also called dense deposit disease, has abnormalities that suggest activation of the alternative pathway. More than 70% of patients have a circulating antibody called C3 nephritic factor, which is an autoantibody that binds to the C3 convertase in the alternative pathway.

C3 nephritic factor binding causes stabilization of the convertase, protecting it from enzymatic degradation and therefore favors persistent C3 activation and hypocomplementemia. It is called dense deposit disease due to deposition of dense material of unknown composition in the glomerular basement membrane.

Membranoproliferative glomerulonephritis -animated quick review

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