A narrative review on C3 glomerulopathy: A rare renal disease

Francesco Paolo Schena, Pasquale Esposito, Michele Rossini

Research output: Contribution to journalReview article

Abstract

In April 2012, a group of nephrologists organized a consensus conference in Cambridge (UK) on type II membranoproliferative glomerulonephritis and decided to use a new terminology, “C3 glomerulopathy” (C3 GP). Further knowledge on the complement system and on kidney biopsy contributed toward distinguishing this disease into three subgroups: dense deposit disease (DDD), C3 glomerulonephritis (C3 GN), and the CFHR5 nephropathy. The persistent presence of microhematuria with or without light or heavy proteinuria after an infection episode suggests the potential onset of C3 GP. These nephritides are characterized by abnormal activation of the complement alternative pathway, abnormal deposition of C3 in the glomeruli, and progression of renal damage to end‐stage kidney disease. The diagnosis is based on studying the complement system, relative genetics, and kidney biopsies. The treatment gap derives from the absence of a robust understanding of their natural outcome. Therefore, a specific treatment for the different types of C3 GP has not been established. Recommendations have been obtained from case series and observational studies because no randomized clinical trials have been conducted. Current treatment is based on corticosteroids and antiproliferative drugs (cyclophosphamide, mycophenolate mofetil), monoclonal antibodies (rituximab) or complement inhibitors (eculizumab). In some cases, it is suggested to include sessions of plasma exchange.

Original languageEnglish
Article number525
JournalInternational Journal of Molecular Sciences
Volume21
Issue number2
DOIs
Publication statusPublished - Jan 2 2020

Fingerprint

Rare Diseases
complement
Membranoproliferative Glomerulonephritis
Biopsy
Complement Inactivating Agents
Kidney
kidneys
Mycophenolic Acid
Alternative Complement Pathway
nephritis
Plasma Exchange
Monoclonal antibodies
kidney diseases
Nephritis
glomerulus
corticosteroids
Kidney Diseases
Terminology
Glomerulonephritis
Proteinuria

Keywords

  • C3 glomerulonephritis
  • C3 glomerulopathy
  • CFHR5 nephropathy
  • Dense deposits disease

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry

Cite this

A narrative review on C3 glomerulopathy : A rare renal disease. / Schena, Francesco Paolo; Esposito, Pasquale; Rossini, Michele.

In: International Journal of Molecular Sciences, Vol. 21, No. 2, 525, 02.01.2020.

Research output: Contribution to journalReview article

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