Anti-factor B antibodies and acute postinfectious GN in children

S Chauvet, R Berthaud, M Devriese… - Journal of the …, 2020 - journals.lww.com
Background The pathophysiology of the leading cause of pediatric acute nephritis, acute
postinfectious GN, including mechanisms of the pathognomonic transient complement …

Longitudinal change in proteinuria and kidney outcomes in C3 glomerulopathy

F Caravaca-Fontán, M Díaz-Encarnación… - Nephrology Dialysis …, 2022 - academic.oup.com
Introduction The association between a change in proteinuria over time and its impact on
kidney prognosis has not been analysed in complement component 3 (C3) glomerulopathy …

Loss of decay-accelerating factor triggers podocyte injury and glomerulosclerosis

A Angeletti, C Cantarelli, A Petrosyan… - Journal of Experimental …, 2020 - rupress.org
Kidney glomerulosclerosis commonly progresses to end-stage kidney failure, but
pathogenic mechanisms are still poorly understood. Here, we show that podocyte …

Treatment of rare inflammatory kidney diseases: drugs targeting the terminal complement pathway

M Anliker-Ort, J Dingemanse, J Van den Anker… - Frontiers in …, 2020 - frontiersin.org
The complement system comprises the frontline of the innate immune system. Triggered by
pathogenic surface patterns in different pathways, the cascade concludes with the formation …

[HTML][HTML] Efficacy and safety of iptacopan in patients with C3 glomerulopathy

E Wong, C Nester, T Cavero, A Karras… - Kidney international …, 2023 - Elsevier
Introduction Complement 3 glomerulopathy (C3G) is a rare inflammatory kidney disease
mediated by dysregulation of the alternative complement pathway. No targeted therapy …

A narrative review on C3 glomerulopathy: A rare renal disease

FP Schena, P Esposito, M Rossini - International journal of molecular …, 2020 - mdpi.com
In April 2012, a group of nephrologists organized a consensus conference in Cambridge
(UK) on type II membranoproliferative glomerulonephritis and decided to use a new …

[HTML][HTML] Clinical safety and efficacy of pegcetacoplan in a phase 2 study of patients with C3 glomerulopathy and other complement-mediated glomerular diseases

BP Dixon, LA Greenbaum, L Huang, S Rajan… - Kidney International …, 2023 - Elsevier
Introduction Dysregulated complement activation is likely the primary driver of disease in C3
glomerulopathy (C3G) and contributes to other complement-mediated diseases, including …

A family affair: addressing the challenges of factor H and the related proteins

F Poppelaars, E Goicoechea de Jorge… - Frontiers in …, 2021 - frontiersin.org
Inflammation is a common denominator of diseases. The complement system, an intrinsic
part of the innate immune system, is a key driver of inflammation in numerous disorders …

Results from a nationwide retrospective cohort measure the impact of C3 and soluble C5b-9 levels on kidney outcomes in C3 glomerulopathy

S Chauvet, JJ Hauer, F Petitprez, M Rabant… - Kidney international, 2022 - Elsevier
C3 glomerulopathy (C3G) is a rare complement-mediated disease. Specific treatments are
not yet available and factors predictive of kidney survival such as age, kidney function and …

Complement and the prothrombotic state

CQ Schmidt, H Schrezenmeier… - Blood, The Journal of …, 2022 - ashpublications.org
In 2007 and 2009, the regulatory approval of the first-in-class complement inhibitor
eculizumab revolutionized the clinical management of 2 rare, life-threatening clinical …