C4 glomerulopathy natural history

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

If left untreated, C4 glomerulopathy may progress to develop renal failure.

Natural History, Complications, and Prognosis

Natural History

The symptoms of C4 glomerulopathy usually start with symptoms such as proteinuria, hematuria, edema, renal insufficiency hypocomplementemia, and hypertension^[1].

Complications

Common complications of C4 glomerulopathy include^[2]^[3]:

Renal failure

Prognosis

Depending on the extent of the disease at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor^[4].

References

↑ Appel GB, Cook HT, Hageman G, Jennette JC, Kashgarian M, Kirschfink M; et al. (2005). “Membranoproliferative glomerulonephritis type II (dense deposit disease): an update”. J Am Soc Nephrol. 16 (5): 1392–403. doi:10.1681/ASN.2005010078. PMID 15800116.
↑ Garam N, Prohászka Z, Szilágyi Á, Aigner C, Schmidt A, Gaggl M; et al. (2019). “C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy”. Orphanet J Rare Dis. 14 (1): 247. doi:10.1186/s13023-019-1237-8. PMC 6839100 Check |pmc= value (help). PMID 31703608.
↑ Ali, Arshad; Schlanger, Lynn; Nasr, Samih H.; Sethi, Sanjeev; Gorbatkin, Steven M. (2016). “Proliferative C4 Dense Deposit Disease, Acute Thrombotic Microangiopathy, a Monoclonal Gammopathy, and Acute Kidney Failure”. American Journal of Kidney Diseases. 67 (3): 479–482. doi:10.1053/j.ajkd.2015.10.020. ISSN 0272-6386.
↑ Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K; et al. (1993). “GeneReviews®”. PMID 20301598.

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[pmid15800116-1] Appel GB, Cook HT, Hageman G, Jennette JC, Kashgarian M, Kirschfink M; et al. (2005). “Membranoproliferative glomerulonephritis type II (dense deposit disease): an update”. J Am Soc Nephrol. 16 (5): 1392–403. doi:10.1681/ASN.2005010078. PMID 15800116.

[pmid31703608-2] Garam N, Prohászka Z, Szilágyi Á, Aigner C, Schmidt A, Gaggl M; et al. (2019). “C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy”. Orphanet J Rare Dis. 14 (1): 247. doi:10.1186/s13023-019-1237-8. PMC 6839100 Check |pmc= value (help). PMID 31703608.

[AliSchlanger2016-3] Ali, Arshad; Schlanger, Lynn; Nasr, Samih H.; Sethi, Sanjeev; Gorbatkin, Steven M. (2016). “Proliferative C4 Dense Deposit Disease, Acute Thrombotic Microangiopathy, a Monoclonal Gammopathy, and Acute Kidney Failure”. American Journal of Kidney Diseases. 67 (3): 479–482. doi:10.1053/j.ajkd.2015.10.020. ISSN 0272-6386.

[pmid20301598-4] Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K; et al. (1993). “GeneReviews®”. PMID 20301598.

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