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Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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C4 glomerulopathy causes

Introduction

Overview

Overview

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

Causes

Causes

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).
  • 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).
References

References

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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.

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]