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Complement 3 glomerulopathy
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
Prevalence
0.2–0.3 / 100 000
660–1,000
US Estimated
900–1,350
Europe Estimated
Age of Onset
All ages
ICD-10
Typically coded under
N04.8
Inheritance Pattern
Autosomal dominant
In some cases
Autosomal recessive
In some cases
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
5 Facts you should know
FACT
The term C3 glomerulopathy was adopted by expert consensus in 2013 to define a group of rare kidney diseases driven by dysregulation of the complement cascade
FACT
C3 glomerulopathy is characterized by accumulation of the C3 component of complement in renal tissue
FACT
There are 2 major subgroups of C3 glomerulopathy — dense deposit disease (DDD) and C3 glomerulonephritis (C3GN)
FACT
About 50% of patients with C3G progress to kidney failure within 10 years of diagnosis
FACT
5
Factor B, factor D, and properdin (factor P) are specific components of the alternative pathway of complement activation
Interest over time
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Common signs & symptoms
Symptoms vary but commonly include:
- Proteinuria
- Hematuria
- Swelling (edema)
- High blood pressure
- Fatigue
- Reduced kidney function
Some patients develop:
- Nephrotic syndrome
- Progressive chronic kidney disease
- End-stage kidney disease
Current treatments
There is no universally effective cure, and treatment focuses on slowing disease progression.
Blood pressure control
- ACE inhibitors or ARBs
Immunosuppressive therapy in selected cases
Complement-targeting therapies (investigational or emerging)
Supportive care for kidney disease
Dialysis or kidney transplantation in advanced cases
New therapies targeting the complement system are currently under investigation.
References:
Martín B, Smith RJH. C3 Glomerulopathy. In: GeneReviews®. University of Washington, Seattle; 2018. — Describes C3G as a complement-mediated kidney disease caused by dysregulation of the alternative complement pathway and notes that inheritance is complex and only occasionally follows dominant or recessive patterns.Smith RJH, Appel GB, Blom AM, et al. C3 glomerulopathy—understanding a rare complement-driven renal disease. Nat Rev Nephrol. 2019;15:129–143. — Review discussing complement dysregulation and the role of genetic variants in complement-related genes in C3G.Piras R, et al. CFH and CFHR copy number variations in C3 glomerulopathy. Front Genet. 2021;12:670727. — Highlights the contribution of complement regulatory genes such as CFH and CFHR variants to C3G pathogenesis.Zhao W, et al. Genetic analysis of the complement pathway in C3 glomerulopathy. Nephrol Dial Transplant. 2018;33(11):1919–1927. — Reports variants in complement pathway genes including CFH, CFI, CD46, and C3 in affected patients. Heidenreich K, et al. C3 glomerulopathy: a kidney disease mediated by complement dysregulation. Front Nephrol. 2024. — Review describing the disease as caused by dysregulation of the alternative complement pathway leading to C3 deposition in glomeruli.