Micrograph of glomerulus in membranoproliferative glomerulonephritis with increased mesangial matrix and increased mesangial cellularity.Kidney biopsy.PAS stain.
Membranoproliferative glomerulonephritis (MPGN) is a type ofglomerulonephritis caused by deposits in thekidney glomerularmesangium and basement membrane (GBM) thickening,[2] activating thecomplement system and damaging the glomeruli.
MPGN accounts for approximately 4% of primary renal causes ofnephrotic syndrome in children and 7% in adults.[3]
It should not be confused withmembranous glomerulonephritis, a condition in which the basement membrane is thickened, but the mesangium is not.
Type I, the most common by far, is caused byimmune complexes depositing in the kidney. It is characterised by subendothelial and mesangial immune deposits.[citation needed]
Type II is today more commonly known asdense deposit disease (DDD).[5] Most cases of dense deposit disease do not show a membranoproliferative pattern.[6] It forms a continuum withC3 glomerulonephritis; together they make up the two major subgroups ofC3 glomerulopathy.[7]
DDD is associated with deposition of complement C3 within the glomeruli with little or no staining for immunoglobulin. The presence of C3 without significant immunoglobulin suggested to early investigators that DDD was due to abnormal activation of the complement alternative pathway (AP). There is now strong evidence that DDD is caused by uncontrolled AP activation.[10]
Spontaneous remissions of MPGN II are rare; approximately half of those affected with MPGN II will progress toend stage renal disease within ten years.[11]
Type III is very rare, it is characterized by a mixture of subepithelial and subendothelial immune and/or complement deposits. These deposits elicit an immune response, causing damage to cells and structures within their vicinity. Has similar pathological findings of Type I disease.[13]
Primary MPGN is treated with steroids, plasma exchange and other immunosuppressive drugs.Secondary MPGN is treated by treating the associated infection, autoimmune disease or neoplasms.Pegylated interferon andribavirin are useful in reducing viral load.[19]
^West CD, McAdams AJ (March 1998). "Glomerular paramesangial deposits: association with hypocomplementemia in membranoproliferative glomerulonephritis types I and III".Am. J. Kidney Dis.31 (3):427–34.doi:10.1053/ajkd.1998.v31.pm9506679.PMID9506679.
^(reviewed in Appel et al., 2005; Smith et al., 2007). Smith, R. J. ., Harris, C. L., & Pickering, M. C. (2011). Dense Deposit Disease. Molecular Immunology, 48(14), 1604–1610.http://doi.org/10.1016/j.molimm.2011.04.005/
^Swainson CP, Robson JS, Thomson D, MacDonald MK (1983). "Mesangiocapillary glomerulonephritis: a long-term study of 40 cases".J. Pathol.141 (4):449–68.doi:10.1002/path.1711410404.PMID6363655.S2CID25434508.
^Colville D, Guymer R, Sinclair RA, Savige J (2003). "Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease")".Am. J. Kidney Dis.42 (2): E2–5.doi:10.1016/S0272-6386(03)00665-6.PMID12900843.
^Pickering, M. C., D’Agati, V. D., Nester, C. M., Smith, R. J., Haas, M., Appel, G. B., … Cook, H. T. (2013). C3 glomerulopathy: consensus report. Kidney International, 84(6), 1079–1089.http://doi.org/10.1038/ki.2013.377
^Neary J, Dorman A, Campbell E, Keogan M, Conlon P (July 2002). "Familial membranoproliferative glomerulonephritis type III".Am. J. Kidney Dis.40 (1): e1.1–e1.6.doi:10.1053/ajkd.2002.33932.PMID12087587.
