KEGG   DISEASE: Membranoproliferative glomerulonephritisHelp
Entry
H01726                      Disease                                

Name
Membranoproliferative glomerulonephritis
  Subgroup
Dense deposit disease (DDD)
C3 glomerulonephritis (C3GN)
Immunoglobulin-mediated membranoproliferative glomerulonephritis
Description
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.
Category
Immune system disease; Urinary system disease
Brite
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
Pathway
hsa04610  Complement and coagulation cascades
Gene
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
Reference
  Authors
Lionaki S, Gakiopoulou H, Boletis JN
  Title
Understanding the complement-mediated glomerular diseases: focus on membranoproliferative glomerulonephritis and C3 glomerulopathies.
  Journal
APMIS 124:725-35 (2016)
DOI:10.1111/apm.12566
Reference
  Authors
Salvadori M, Rosso G
  Title
Reclassification of membranoproliferative glomerulonephritis: Identification of a new GN: C3GN.
  Journal
World J Nephrol 5:308-20 (2016)
DOI:10.5527/wjn.v5.i4.308
Reference
  Authors
Floege J, Amann K
  Title
Primary glomerulonephritides.
  Journal
Lancet 387:2036-48 (2016)
DOI:10.1016/S0140-6736(16)00272-5

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