C4 glomerulopathy classification

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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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