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==Overview==
==Overview==
C4 glomerulopathy may be classified based on complement deposition into Dense Deposit Disease (DDD) and C4 Glomerulonephritis (C4GN).
C4 glomerulopathy may be classified based on complement deposition into dense deposit disease and C4 glomerulonephritis.


==Classification==
==Classification==
*[[C4 (complement)|C4]] [[glomerulopathy]] is categorized as a variant of [[MPGN]]<ref name="pmid26704376">{{cite journal| author=Ali A, Schlanger L, Nasr SH, Sethi S, Gorbatkin SM| title=Proliferative C4 Dense Deposit Disease, Acute Thrombotic Microangiopathy, a Monoclonal Gammopathy, and Acute Kidney Failure. | journal=Am J Kidney Dis | year= 2016 | volume= 67 | issue= 3 | pages= 479-82 | pmid=26704376 | doi=10.1053/j.ajkd.2015.10.020 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26704376  }} </ref><ref name="pmid24552345">{{cite journal| author=Sethi S, Sullivan A, Smith RJ| title=C4 dense-deposit disease. | journal=N Engl J Med | year= 2014 | volume= 370 | issue= 8 | pages= 784-6 | pmid=24552345 | doi=10.1056/NEJMc1309449 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24552345  }} </ref>.
*[[C4 (complement)|C4]] [[glomerulopathy]] is categorized as a variant of [[MPGN]]<ref name="pmid26704376">{{cite journal| author=Ali A, Schlanger L, Nasr SH, Sethi S, Gorbatkin SM| title=Proliferative C4 Dense Deposit Disease, Acute Thrombotic Microangiopathy, a Monoclonal Gammopathy, and Acute Kidney Failure. | journal=Am J Kidney Dis | year= 2016 | volume= 67 | issue= 3 | pages= 479-82 | pmid=26704376 | doi=10.1053/j.ajkd.2015.10.020 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26704376  }} </ref><ref name="pmid24552345">{{cite journal| author=Sethi S, Sullivan A, Smith RJ| title=C4 dense-deposit disease. | journal=N Engl J Med | year= 2014 | volume= 370 | issue= 8 | pages= 784-6 | pmid=24552345 | doi=10.1056/NEJMc1309449 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24552345  }} </ref>.
*Recently, Sethi et al. have defined a new term “C4d glomerulopathy”
*Recently, Sethi et al. have defined a new term “C4d glomerulopathy”
*C4 Glomerulopathy may be classified into 2 main subtypes based on the appearance of complement deposition in the glomerular basement membrane on Electron Microscopy:
*C4 Glomerulopathy may be classified into 2 main subtypes based on the appearance of complement deposition in the glomerular basement membrane on electron microscopy:
**'''Dense deposit disease (DDD)'''
**'''Dense deposit disease (DDD)'''
***Presence of C4 granular or osmiophilic deposition along the basement membrane on immunofluorescence or immunohistochemistry.
***Presence of C4 granular or osmiophilic deposition along the basement membrane on immunofluorescence or immunohistochemistry.

Latest revision as of 04:37, 8 August 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3]

Overview

C4 glomerulopathy may be classified based on complement deposition into dense deposit disease and C4 glomerulonephritis.

Classification

  • C4 glomerulopathy is categorized as a variant of MPGN[1][2].
  • Recently, Sethi et al. have defined a new term “C4d glomerulopathy”
  • C4 Glomerulopathy may be classified into 2 main subtypes based on the appearance of complement deposition in the glomerular basement membrane on electron microscopy:
    • Dense deposit disease (DDD)
      • Presence of C4 granular or osmiophilic deposition along the basement membrane on immunofluorescence or immunohistochemistry.
    • C4 Glomerulonephritis (C4GN)
      • Isolated deposition of C4.

References

  1. Ali A, Schlanger L, Nasr SH, Sethi S, Gorbatkin SM (2016). "Proliferative C4 Dense Deposit Disease, Acute Thrombotic Microangiopathy, a Monoclonal Gammopathy, and Acute Kidney Failure". Am J Kidney Dis. 67 (3): 479–82. doi:10.1053/j.ajkd.2015.10.020. PMID 26704376.
  2. Sethi S, Sullivan A, Smith RJ (2014). "C4 dense-deposit disease". N Engl J Med. 370 (8): 784–6. doi:10.1056/NEJMc1309449. PMID 24552345.

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