C4 glomerulopathy pathophysiology: Difference between revisions
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==Overview== | ==Overview== | ||
It is thought that C4 glomerulopathy is the result of the activation of immune complex glomerulonephritis or lectin pathway of the complement. | |||
==Pathophysiology== | ==Pathophysiology== | ||
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*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. | *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. | *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. | *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<ref name="pmid25991041">{{cite journal| author=Sethi S, Nasr SH, De Vriese AS, Fervenza FC| title=C4d as a Diagnostic Tool in Proliferative GN. | journal=J Am Soc Nephrol | year= 2015 | volume= 26 | issue= 11 | pages= 2852-9 | pmid=25991041 | doi=10.1681/ASN.2014040406 | pmc=4625660 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25991041 }} </ref><ref name="pmid26154922">{{cite journal| author=Larsen CP, Messias NC, Walker PD, Fidler ME, Cornell LD, Hernandez LH | display-authors=etal| title=Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits. | journal=Kidney Int | year= 2015 | volume= 88 | issue= 4 | pages= 867-73 | pmid=26154922 | doi=10.1038/ki.2015.195 | pmc=4687465 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26154922 }} </ref><ref name="pmid14747370">{{cite journal| author=Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB | display-authors=etal| title=The classification of glomerulonephritis in systemic lupus erythematosus revisited. | journal=J Am Soc Nephrol | year= 2004 | volume= 15 | issue= 2 | pages= 241-50 | pmid=14747370 | doi=10.1097/01.asn.0000108969.21691.5d | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14747370 }} </ref><ref name="pmid21959138">{{cite journal| author=Batal I, Liang K, Bastacky S, Kiss LP, McHale T, Wilson NL | display-authors=etal| title=Prospective assessment of C4d deposits on circulating cells and renal tissues in lupus nephritis: a pilot study. | journal=Lupus | year= 2012 | volume= 21 | issue= 1 | pages= 13-26 | pmid=21959138 | doi=10.1177/0961203311422093 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21959138 }} </ref><ref name="pmid24578331">{{cite journal| author=Espinosa M, Ortega R, Sánchez M, Segarra A, Salcedo MT, González F | display-authors=etal| title=Association of C4d deposition with clinical outcomes in IgA nephropathy. | journal=Clin J Am Soc Nephrol | year= 2014 | volume= 9 | issue= 5 | pages= 897-904 | pmid=24578331 | doi=10.2215/CJN.09710913 | pmc=4011455 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24578331 }} </ref>. | ||
==References== | ==References== | ||
Latest revision as of 05:29, 6 August 2020
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C4 glomerulopathy Microchapters |
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Diagnosis |
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Case Studies |
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C4 glomerulopathy pathophysiology On the Web |
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American Roentgen Ray Society Images of C4 glomerulopathy pathophysiology |
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Risk calculators and risk factors for C4 glomerulopathy pathophysiology |
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.