DR Medhat MRCP

N.B :
C3 glomerulopathy
Isolated deposition of C3, often with similar features to type I, but with
no immunoglobulin deposition.
Mesangial, subendothelial, subepithelial, and/or intramembranous deposits
on EM
Associated with genetic or acquired abnormalities of complement regulation.
Serum C3 nephritic factor is positive .
Such diseases, including C3GN and Dense Deposit Disease, have been termed
C3 glomerulopathies and should suggest investigation of the alternative
complement pathway (e.g. functional assays, genetic mutation analysis, and
autoantibody screening)
Management of MCGN :
Exclude & treat 2ndry causes prior to treatment
TTT of HCV –related MCGN
A) Antiviral TTT(KDIGO guidelines): :
• CKD stage 1 – 2: Peginterferon alfa and Ribavirin.
• CKD stages 3 – 4: Peginterferon alfa monotherapy.
• Haemodialysis: Standard interferon alfa.
• Ribavirin ( ± ESA support) may be considered for all stages in specialized
centres.
B) TTT of severe active disease (e.g RPGN) :
• Plasma exchange: x 3/week for 2 – 3 weeks to remove cryoglobulins
• Rituximab : 375mg/m 2 weekly for 4 weeks to stop further B cell production
(also an option if intolerant/resistant to interferon alfa/ribavirin).
• Corticosteroids : as methylprednisolone 0.5 – 1g/day for 3 days, then oral.
• Cyclophosphamide 1.5 – 2.0mg/kg daily orally for 2 – 4 months
• Novel direct-acting antivirals (Boceprevir or Telaprevir) + PEG-IFN- α and
ribavirin to increase the viral response in genotype 1 HCV infection (only if
RFT is normal)
• Low-dose IL-2 (under research)
Immunosuppressants (for nephritic syndrome & RPGN) :
• Corticosteroids (tapering prednisolone started at 1mg/kg/day).
• Corticosteroids ± cyclophosphamide 1.5 – 2mg/kg/day for ≤ 6 months.
• Corticosteroids ± MMF for ≤ 6 months (less toxic alternative).
General measures of nephritic syndrome.
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