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Update on C3 Glomerulopathy.
- Source :
-
Advances in kidney disease and health [Adv Kidney Dis Health] 2024 May; Vol. 31 (3), pp. 223-233. - Publication Year :
- 2024
-
Abstract
- C3 glomerulopathy (C3G) is a rare disorder marked by deposition of C3 in the glomerulus, resulting in damage to the glomerular filtration unit and presenting with features of the nephritic and nephrotic syndromes. Fundamentally, C3G is caused by dysregulation of the alternative pathway of the complement cascade, either due to genetic variants or acquired humoral factors. Despite significant advances in recent years in the understanding of the underlying mechanisms and culprit lesions that result in the development of C3G, treatment options remain severely limited, and the prognosis is often poor. Fortunately, a number of anticomplement therapies are emerging from the drug development pipeline, with several in late-stage testing in patients with C3G, and there is hope that we will soon have more targeted options for managing patients with this devastating disease. In this review, we provide an overview of C3G, as well as summarizing the evidence for current treatments and detailing the clinical trials that are currently underway.<br /> (Copyright © 2024. Published by Elsevier Inc.)
- Subjects :
- Humans
Kidney Glomerulus pathology
Kidney Glomerulus metabolism
Complement Pathway, Alternative genetics
Complement Pathway, Alternative drug effects
Glomerulonephritis pathology
Glomerulonephritis metabolism
Glomerulonephritis genetics
Glomerulonephritis immunology
Glomerulonephritis therapy
Glomerulonephritis diagnosis
Nephrotic Syndrome pathology
Nephrotic Syndrome genetics
Nephrotic Syndrome metabolism
Nephrotic Syndrome diagnosis
Nephrotic Syndrome therapy
Complement C3 metabolism
Complement C3 genetics
Complement C3 immunology
Subjects
Details
- Language :
- English
- ISSN :
- 2949-8139
- Volume :
- 31
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Advances in kidney disease and health
- Publication Type :
- Academic Journal
- Accession number :
- 39004462
- Full Text :
- https://doi.org/10.1053/j.akdh.2024.05.002