C4 glomerulopathy causes

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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

C4 glomerulopathy may be caused by mutation of complement factor,anti-factor H, anti-factor B, or C4 nephritic factor.

Common causes of is not yet known but C4 glomerulopathy is analogous to C3 glomerulopathy and the causes may include^[1]^[2]^[3]^[4]^[5]:

C4 nephritic factor-an autoantibody against C4 can cause complement dysregulation against immune complex-myeloproliferative glomerulonephritic patients.
Mutation of complement factor H (CFH).
- CFH is a small glycoprotein that is produced in the liver and circulates freely in the blood plasma.
- Autoantibodies against CFH.
Anti-Factor H, anti-Factor B, and C3- or C4 nephritic factors are frequently seen in patients with complement-mediated renal disorders.
Mutation of membrane cofactor protein (MCP).

↑ Rodríguez de Córdoba S, Esparza-Gordillo J, Goicoechea de Jorge E, Lopez-Trascasa M, Sánchez-Corral P (June 2004). "The human complement factor H: functional roles, genetic variations and disease associations". Mol. Immunol. 41 (4): 355–67. doi:10.1016/j.molimm.2004.02.005. PMID 15163532.
↑ Noris M, Donadelli R, Remuzzi G (June 2018). "Autoimmune abnormalities of the alternative complement pathway in membranoproliferative glomerulonephritis and C3 glomerulopathy". Pediatr. Nephrol. doi:10.1007/s00467-018-3989-0. PMID 29948306.
↑ Marinozzi MC, Roumenina LT, Chauvet S, Hertig A, Bertrand D, Olagne J; et al. (2017). "Anti-Factor B and Anti-C3b Autoantibodies in C3 Glomerulopathy and Ig-Associated Membranoproliferative GN". J Am Soc Nephrol. 28 (5): 1603–1613. doi:10.1681/ASN.2016030343. PMC 5407719. PMID 28096309.
↑ Zhang Y, Meyer NC, Fervenza FC, Lau W, Keenan A, Cara-Fuentes G; et al. (2017). "C4 Nephritic Factors in C3 Glomerulopathy: A Case Series". Am J Kidney Dis. 70 (6): 834–843. doi:10.1053/j.ajkd.2017.07.004. PMC 5701858. PMID 28838767.
↑ Blanc C, Togarsimalemath SK, Chauvet S, Le Quintrec M, Moulin B, Buchler M; et al. (2015). "Anti-factor H autoantibodies in C3 glomerulopathies and in atypical hemolytic uremic syndrome: one target, two diseases". J Immunol. 194 (11): 5129–38. doi:10.4049/jimmunol.1402770. PMID 25917093.