C4 glomerulopathy pathophysiology
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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
It is thought that C4 glomerulopathy is the result of the activation of immune complex glomerulonephritis or lectin pathway of the complement.
Pathophysiology
- The generation of the classical pathway of complement occurs by the immune complex.
- Immune complex glomerulonephritis, such as lupus, frequently activates the classical pathway by binding C1q to the antibody-containing immune complexes, which can result in glomerular deposition of immunoglobulins, C3, C1q, and C4d.
- C4 activation can also ensue by the lectin pathway of complement.
- In the lectin pathway, mannose-binding lectins bind to mannose located on the carbohydrates structure or microbial membranes, which activate C4 (and therefore bypass C1q), resulting in the formation of the classical pathway C3 convertase.
- Glomerular C4d, is a split creation of C4, is suggestive of C4 activation as a result of activation of either the lectin pathway of the classical complement pathway.
- In C4 glomerulopathy, bright streak for C4d indicates mesangial/capillary wall deposits. C4d deposits are present in a few cases of IgA nephropathy and post-streptococcal glomerulonephritis[1][2][3][4][5].
References
- ↑ Sethi S, Nasr SH, De Vriese AS, Fervenza FC (2015). "C4d as a Diagnostic Tool in Proliferative GN". J Am Soc Nephrol. 26 (11): 2852–9. doi:10.1681/ASN.2014040406. PMC 4625660. PMID 25991041.
- ↑ Larsen CP, Messias NC, Walker PD, Fidler ME, Cornell LD, Hernandez LH; et al. (2015). "Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits". Kidney Int. 88 (4): 867–73. doi:10.1038/ki.2015.195. PMC 4687465. PMID 26154922.
- ↑ Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB; et al. (2004). "The classification of glomerulonephritis in systemic lupus erythematosus revisited". J Am Soc Nephrol. 15 (2): 241–50. doi:10.1097/01.asn.0000108969.21691.5d. PMID 14747370.
- ↑ Batal I, Liang K, Bastacky S, Kiss LP, McHale T, Wilson NL; et al. (2012). "Prospective assessment of C4d deposits on circulating cells and renal tissues in lupus nephritis: a pilot study". Lupus. 21 (1): 13–26. doi:10.1177/0961203311422093. PMID 21959138.
- ↑ Espinosa M, Ortega R, Sánchez M, Segarra A, Salcedo MT, González F; et al. (2014). "Association of C4d deposition with clinical outcomes in IgA nephropathy". Clin J Am Soc Nephrol. 9 (5): 897–904. doi:10.2215/CJN.09710913. PMC 4011455. PMID 24578331.