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C3 glomerulopathy — understanding a rare complement-driven renal disease

Lookup NU author(s): Dr Veronique Fremaux-Bacchi, Professor David Kavanagh

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Abstract

© 2019, Springer Nature Limited. The C3 glomerulopathies are a group of rare kidney diseases characterized by complement dysregulation occurring in the fluid phase and in the glomerular microenvironment, which results in prominent complement C3 deposition in kidney biopsy samples. The two major subgroups of C3 glomerulopathy — dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) — have overlapping clinical and pathological features suggestive of a disease continuum. Dysregulation of the complement alternative pathway is fundamental to the manifestations of C3 glomerulopathy, although terminal pathway dysregulation is also common. Disease is driven by acquired factors in most patients — namely, autoantibodies that target the C3 or C5 convertases. These autoantibodies drive complement dysregulation by increasing the half-life of these vital but normally short-lived enzymes. Genetic variation in complement-related genes is a less frequent cause. No disease-specific treatments are available, although immunosuppressive agents and terminal complement pathway blockers are helpful in some patients. Unfortunately, no treatment is universally effective or curative. In aggregate, the limited data on renal transplantation point to a high risk of disease recurrence (both DDD and C3GN) in allograft recipients. Clinical trials are underway to test the efficacy of several first-generation drugs that target the alternative complement pathway.


Publication metadata

Author(s): Smith RJH, Appel GB, Blom AM, Cook HT, D'Agati VD, Fakhouri F, Fremeaux-Bacchi V, Jozsi M, Kavanagh D, Lambris JD, Noris M, Pickering MC, Remuzzi G, de Cordoba SR, Sethi S, Van der Vlag J, Zipfel PF, Nester CM

Publication type: Review

Publication status: Published

Journal: Nature Reviews Nephrology

Year: 2019

Volume: 15

Issue: 3

Pages: 129-143

Print publication date: 01/03/2019

Online publication date: 28/01/2019

Acceptance date: 02/04/2018

ISSN (print): 1759-5061

ISSN (electronic): 1759-507X

Publisher: Nature Publishing Group

URL: https://doi.org/10.1038/s41581-018-0107-2

DOI: 10.1038/s41581-018-0107-2


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