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.
• 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.