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Primary membranoproliferative glomerulonephritis

Disease definition

A rare glomerular disease characterized by a pattern of glomerular injury on kidney biopsy with characteristic light microscopic changes: mesangial hypercellularity, endocapillary proliferation, and thickening of the glomerular basement membrane (GBM). On the basis of immunofluorescence (IF) the disorder is divided into C3 glomerulopathy (C3G) or immunoglobulin-mediated membranoproliferative glomerulonephritis. Through electron microscopy C3G is further divided into Dense deposit disease, with highly electrondense deposits in the glomerular basement membrane, and C3 glomerulonephritis, with mesangial, intramembranous, subendothelial and subepithelial deposits. Secondary causes (autoimmune, infectious, malignancies) are excluded.

ORPHA:54370

Classification level: Disorder
  • Synonym(s):
    • Mesangiocapillary glomerulonephritis
    • Primary MPGN
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: N03.5
  • OMIM: 305800  609814  614809  615008
  • UMLS: C0017662
  • MeSH: D015432
  • GARD: 11982
  • MedDRA: 10018370
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