C4 glomerulopathy other diagnostic studies

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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

A kidney biopsy may be helpful in the diagnosis of C4 glomerulopathy. Findings diagnostic of C4 glomerulopathy include glomerular capillary walls thickening, large subendothelial osmiophilic dense deposits, and ribbonlike material on the glomerular basement membrane.

Other Diagnostic Studies

A kidney biopsy may be helpful in the diagnosis of C4 glomerulopathy. Findings suggestive of C4 glomerulopathy include[1][2][3][4][5]:

  • Membranoproliferative glomerulonephritis, with mesangial and endocapillary hypercellularity.
  • The glomerular capillary walls thickening.
  • Periodic acid–Schiff stain finding of ribbonlike material on the glomerular basement membrane.
  • Immunofluorescence microscopy indicates no glomerular staining for IgA, IgM, IgG, C3, C1q, kappa or lambda light chains.
  • Electron microscopy is favorable for large subendothelial osmiophilic dense deposits lining the glomerular basement membrane.
  • C4d glomerular staining help as a marker for immune complex-mediated glomerulonephritis.
  • Positive C4d can also assist as a marker for glomerulopathy resulting from activation of the lectin pathway.

References

  1. Sethi S, Fervenza FC (2012). "Membranoproliferative glomerulonephritis--a new look at an old entity". N Engl J Med. 366 (12): 1119–31. doi:10.1056/NEJMra1108178. PMID 22435371.
  2. Zhang Y, Meyer NC, Wang K, Nishimura C, Frees K, Jones M; et al. (2012). "Causes of alternative pathway dysregulation in dense deposit disease". Clin J Am Soc Nephrol. 7 (2): 265–74. doi:10.2215/CJN.07900811. PMC 3280037. PMID 22223606.
  3. Sahin OZ, Gurses S, Taslı F, Yavas H, Ersoy R, Uzum A; et al. (2013). "Glomerular c4d staining can be an indicator of disease activity in lupus nephritis". Ren Fail. 35 (2): 222–5. doi:10.3109/0886022X.2012.743916. PMID 23176021.
  4. 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.
  5. 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.

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