Display options
Share it on

Indian J Nephrol. 2014 Nov;24(6):339-48. doi: 10.4103/0971-4065.134089.

Current concepts in C3 glomerulopathy.

Indian journal of nephrology

S Thomas, D Ranganathan, L Francis, K Madhan, G T John

Affiliations

  1. Department of Renal Medicine, Royal Brisbane and Women's Hospital, Brisbane, Australia.
  2. Department of Pathology, Royal Brisbane and Women's Hospital, Brisbane, Australia.
  3. Department of Renal Medicine, Hervey Bay Hospital, Hervey Bay, Australia.

PMID: 25484526 PMCID: PMC4244712 DOI: 10.4103/0971-4065.134089

Abstract

Complement component 3 glomerulopathy (C3G) is a recently defined entity comprising of dense deposit disease and C3 glomerulonephritis. The key histological feature is the presence of isolated C3 deposits without immunoglobulins. Often masqueradng as some of the common glomerulonephritides this is a prototype disorder occurring from dysregulated alternate complement pathway with recently identified genetic defects and autoantibodies. We review the pathophysiology, clinical features, and diagnostic and treatment strategies.

Keywords: Alternate complement pathway; complement component 3 glomerulonephritis; complement component 3 glomerulopathy; complements; dense deposit disease

References

  1. Mol Immunol. 2011 Aug;48(14):1604-10 - PubMed
  2. Nat Genet. 2006 Oct;38(10):1173-7 - PubMed
  3. Kidney Int. 2013 Feb;83(2):293-9 - PubMed
  4. Kidney Int. 2006 Jul;70(1):42-50 - PubMed
  5. J Clin Invest. 1964 Aug;43:1507-17 - PubMed
  6. Kidney Int. 1975 Apr;7(4):204-15 - PubMed
  7. J Urol Nephrol (Paris). 1962 Jan-Feb;68:116-22 - PubMed
  8. Am J Kidney Dis. 2010 Nov;56(5):977-82 - PubMed
  9. Proc R Soc Med. 1972 Jul;65(7):591 - PubMed
  10. Am J Kidney Dis. 2002 Jul;40(1):E1 - PubMed
  11. Kidney Int. 2014 Feb;85(2):450-6 - PubMed
  12. Clin J Am Soc Nephrol. 2012 May;7(5):748-56 - PubMed
  13. Nat Rev Nephrol. 2012 Nov;8(11):634-42 - PubMed
  14. Dis Model Mech. 2011 Nov;4(6):721-6 - PubMed
  15. J Med Genet. 2007 Mar;44(3):193-9 - PubMed
  16. Kidney Int. 2009 Jun;75(11):1230-4 - PubMed
  17. Mol Immunol. 2004 May;41(1):81-4 - PubMed
  18. N Engl J Med. 1976 Feb 26;294(9):461-5 - PubMed
  19. Lancet. 2010 Sep 4;376(9743):794-801 - PubMed
  20. J Exp Med. 1960 Feb 1;111:201-15 - PubMed
  21. Am J Kidney Dis. 2012 Dec;60(6):1039-43 - PubMed
  22. J Am Soc Nephrol. 2013 Nov;24(11):1820-9 - PubMed
  23. Nephrol Dial Transplant. 1994;9(12):1747-50 - PubMed
  24. Clin Exp Immunol. 1983 Oct;54(1):253-8 - PubMed
  25. Pediatr Nephrol. 2000 Apr;14(4):322-4 - PubMed
  26. Rev Hematol. 1958 Apr-Jun;13(2):239-48 - PubMed
  27. J Am Soc Nephrol. 2012 Jul;23(7):1229-37 - PubMed
  28. Trends Immunol. 2008 Aug;29(8):380-7 - PubMed
  29. J Am Soc Nephrol. 2007 Sep;18(9):2447-56 - PubMed
  30. Am J Kidney Dis. 2000 Apr;35(4):749-52 - PubMed
  31. J Clin Invest. 1973 Apr;52(4):896-904 - PubMed
  32. Clin J Am Soc Nephrol. 2011 Jun;6(6):1436-46 - PubMed
  33. Am J Med. 1975 Jun;58(6):749-64 - PubMed
  34. Am J Kidney Dis. 2001 Jun;37(6):1120-30 - PubMed
  35. Kidney Int. 1986 Dec;30(6):949-56 - PubMed
  36. Kidney Int. 2009 May;75(9):952-60 - PubMed
  37. Science. 1969 Apr 25;164(3878):436-7 - PubMed
  38. J Am Soc Nephrol. 2005 May;16(5):1392-403 - PubMed
  39. Nephrol Dial Transplant. 1996 Nov;11(11):2309-12 - PubMed
  40. Kidney Int. 1983 May;23(5):749-58 - PubMed
  41. Kidney Int. 2012 Aug;82(4):454-64 - PubMed
  42. J Renin Angiotensin Aldosterone Syst. 2000 Dec;1(4):328-35 - PubMed
  43. J Exp Med. 1974 May 1;139(5):1249-61 - PubMed
  44. Kidney Int. 2012 Aug;82(4):465-73 - PubMed
  45. Kidney Int. 2013 Dec;84(6):1079-89 - PubMed
  46. N Engl J Med. 2012 Mar 22;366(12):1165-6 - PubMed
  47. J Am Soc Nephrol. 2012 Jul;23(7):1155-60 - PubMed
  48. Nat Genet. 2011 Mar 13;43(4):321-7 - PubMed
  49. Pediatr Nephrol. 2012 May;27(5):773-81 - PubMed
  50. Br J Ophthalmol. 1989 Apr;73(4):297-302 - PubMed
  51. J Exp Med. 1962 Jan 1;115:63-82 - PubMed
  52. Transplant Proc. 2006 Mar;38(2):470-2 - PubMed
  53. Am J Transplant. 2011 Jan;11(1):152-5 - PubMed
  54. Lancet. 1999 Jul 31;354(9176):359-64 - PubMed
  55. Mod Pathol. 2007 Jun;20(6):605-16 - PubMed
  56. Am J Kidney Dis. 1994 Dec;24(6):956-63 - PubMed
  57. Kidney Int. 2010 Apr;77(8):721-8 - PubMed
  58. Clin J Am Soc Nephrol. 2009 Jan;4(1):22-32 - PubMed
  59. Eye (Lond). 2001 Jun;15(Pt 3):390-5 - PubMed
  60. Am J Kidney Dis. 2011 Feb;57(2):291-9 - PubMed
  61. J Clin Invest. 2008 Feb;118(2):608-18 - PubMed

Publication Types