KEGG   DISEASE: Membranoproliferative glomerulonephritisHelp
H01726                      Disease                                

Membranoproliferative glomerulonephritis
Dense deposit disease (DDD)
C3 glomerulonephritis (C3GN)
Immunoglobulin-mediated membranoproliferative glomerulonephritis
Membranoproliferative glomerulonephritis (MPGN) represents a pattern of glomerular injury, characterized by mesangial proliferation and expansion, lobularization of the glomerular tufts and double contours. Classification of MPGN has been updated in the 2010s, and is now categorized according to the immunofluorescence microscopy findings. Until recently, the MPGNs have been distinguished according to the histological and ultra structural findings and were classified as MPGN type I, type II and type III. A new terminology that distinguishes immunoglobulin-mediated MPGN (ie, the former type I) from complement-mediated MPGN has been proposed. Immune-complexes mediated MPGN is caused by the deposition of immunocomplexes in the glomeruli. The immunocomplexes activate the classical pathway (CP) of complement and cause the deposition of complement factors or of the membrane attack complex (MAC) in the mesangium and capillary loops. The most frequent underlying disorders associated with immune complex MPGN include chronic infections, autoimmune diseases, and monoclonal gammopathies. The complement-mediated MPGN, termed C3 glomerulopathy, is characterised by defects in the alternative pathway of complement, in particular of factor H, or autoantibodies to complement-regulatory proteins (so-called C3 nephritic factors) leading to increased complement activation. The group includes dense deposit disease (DDD) and C3 glomerulonephritis. The two entities are distinguished on the basis of the immunohistological pattern of C3 and the electron microscopy detection of ribbon-like electron-dense deposits in the glomerular basement membrane in DDD, versus deposits of usual density in C3 glomerulonephritis.
Immune system disease; Urinary system disease
Human diseases [BR:br08402]
 Immune system diseases
  Allergies and autoimmune diseases
   H01726  Membranoproliferative glomerulonephritis
 Urinary system diseases
  Kidney diseases
   H01726  Membranoproliferative glomerulonephritis
Human diseases in ICD-11 classification [BR:br08403]
 21 Symptoms, signs or clinical findings, not elsewhere classified
  Symptoms, signs or clinical findings of the genitourinary system
   Clinical findings in specimens from the urinary system
    MF8Y  Other specified clinical findings in specimens from the urinary system
     H01726  Membranoproliferative glomerulonephritis
BRITE hierarchy
hsa04610  Complement and coagulation cascades
CFH [HSA:3075] [KO:K04004]
CFHR5 [HSA:81494] [KO:K23817]
DGKE [HSA:8526] [KO:K00901]
Other DBs
ICD-11: MF8Y
ICD-10: N00.5
MeSH: D015432
OMIM: 609814 614809 615008
Lionaki S, Gakiopoulou H, Boletis JN
Understanding the complement-mediated glomerular diseases: focus on membranoproliferative glomerulonephritis and C3 glomerulopathies.
APMIS 124:725-35 (2016)
Salvadori M, Rosso G
Reclassification of membranoproliferative glomerulonephritis: Identification of a new GN: C3GN.
World J Nephrol 5:308-20 (2016)
Floege J, Amann K
Primary glomerulonephritides.
Lancet 387:2036-48 (2016)

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