Endothelium structure and function in kidney health and disease

N Jourde-Chiche, F Fakhouri, L Dou, J Bellien… - Nature Reviews …, 2019 - nature.com
The kidney harbours different types of endothelia, each with specific structural and functional
characteristics. The glomerular endothelium, which is highly fenestrated and covered by a …

C3 glomerulopathy—understanding a rare complement-driven renal disease

RJH Smith, GB Appel, AM Blom, HT Cook… - Nature reviews …, 2019 - nature.com
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 …

KDIGO 2021 clinical practice guideline for the management of glomerular diseases

BH Rovin, SG Adler, J Barratt, F Bridoux… - Kidney …, 2021 - kidney-international.org
Glomerular disease, be it primary or secondary, occurring in the setting of systemic
autoimmune diseases, infections, drugs, or malignancy, affects individuals of all ages. In …

Acute glomerulonephritis

S Sethi, AS De Vriese, FC Fervenza - The Lancet, 2022 - thelancet.com
Glomerulonephritis is a heterogeneous group of disorders that present with a combination of
haematuria, proteinuria, hypertension, and reduction in kidney function to a variable degree …

[HTML][HTML] Complement inhibitors in clinical trials for glomerular diseases

PF Zipfel, T Wiech, R Rudnick, S Afonso… - Frontiers in …, 2019 - frontiersin.org
Defective complement action is a cause of several human glomerular diseases including
atypical hemolytic uremic syndrome (aHUS), anti-neutrophil cytoplasmic antibody mediated …

[HTML][HTML] Alternative complement pathway inhibition with iptacopan for the treatment of C3 glomerulopathy-study design of the APPEAR-C3G trial

AS Bomback, D Kavanagh, M Vivarelli, M Meier… - Kidney International …, 2022 - Elsevier
Introduction Complement 3 glomerulopathy (C3G) is a rare kidney disease characterized by
dysregulation of the alternative pathway (AP) of the complement system. About 50% of …

Advances in understanding of pathogenesis and treatment of immune-mediated kidney disease: A review

S Kant, A Kronbichler, P Sharma, D Geetha - American Journal of Kidney …, 2022 - Elsevier
There continues to be rapid advancement in our understanding of the pathogenesis of
immune-mediated kidney disease. This progress has culminated in the development of …

Deposition of the membrane attack complex in healthy and diseased human kidneys

JJE Koopman, MF Van Essen, HG Rennke… - Frontiers in …, 2021 - frontiersin.org
The membrane attack complex—also known as C5b-9—is the end-product of the classical,
lectin, and alternative complement pathways. It is thought to play an important role in the …

Complement-mediated kidney diseases

F Poppelaars, JM Thurman - Molecular Immunology, 2020 - Elsevier
It has long been known that the complement cascade is activated in various forms of
glomerulonephritis. In many of these diseases, immune-complexes deposit in the glomeruli …

C3 glomerulopathy: understanding an ultra‐rare complement‐mediated renal disease

AK Heiderscheit, JJ Hauer… - American Journal of …, 2022 - Wiley Online Library
Abstract C3 glomerulopathy (C3G) describes a pathologic pattern of injury diagnosed by
renal biopsy. It is characterized by the dominant deposition of the third component of …