[HTML][HTML] Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes”(KDIGO) Controversies …

THJ Goodship, HT Cook, F Fakhouri, FC Fervenza… - Kidney international, 2017 - Elsevier
In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G)
complement plays a primary role in disease pathogenesis. Herein we report the outcome of …

Nephritic factors: an overview of classification, diagnostic tools and clinical associations

F Corvillo, M Okrój, P Nozal, M Melgosa… - Frontiers in …, 2019 - frontiersin.org
Nephritic factors comprise a heterogeneous group of autoantibodies against neoepitopes
generated in the C3 and C5 convertases of the complement system, causing its …

Kidney disease caused by dysregulation of the complement alternative pathway: an etiologic approach

AS De Vriese, S Sethi, J Van Praet… - Journal of the …, 2015 - journals.lww.com
Kidney diseases caused by genetic or acquired dysregulation of the complement alternative
pathway (AP) are traditionally classified on the basis of clinical presentation (atypical …

[HTML][HTML] Effectiveness of mycophenolate mofetil in C3 glomerulonephritis

C Rabasco, T Cavero, E Román, J Rojas-Rivera… - Kidney international, 2015 - Elsevier
C3 glomerulonephritis is a clinicopathologic entity defined by the presence of isolated or
dominant deposits of C3 on immunofluorescence. To explore the effect of …

Patterns of clinical response to eculizumab in patients with C3 glomerulopathy

M Le Quintrec, AL Lapeyraque, A Lionet… - American Journal of …, 2018 - Elsevier
Background Cases reports and small series of patients with C3 glomerulopathy have
reported variable efficacy of eculizumab. Study Design Case series of C3 glomerulopathy …

Use of eculizumab in crescentic IgA nephropathy: proof of principle and conundrum?

T Ring, BB Pedersen, G Salkus… - Clinical kidney …, 2015 - academic.oup.com
IgA nephropathy (IgAN) is characterized by a variable clinical course and multifaceted
pathophysiology. There is substantial evidence to suggest that complement activation plays …

C3 Glomerulopathy

M Riedl, P Thorner, C Licht - Pediatric Nephrology, 2017 - Springer
Recent advances in our understanding of the disease pathology of membranoproliferative
glomerulonephritis has resulted in its re-classification as complement C3 glomerulopathy …

Treating C3 glomerulopathy with eculizumab

T Welte, F Arnold, J Kappes, M Seidl, K Häffner… - BMC nephrology, 2018 - Springer
Abstract Background C3 glomerulopathy (C3G) is a rare, but severe glomerular disease with
grim prognosis. The complex pathogenesis is just unfolding, and involves acquired as well …

Update on C3 glomerulopathy

TD Barbour, MM Ruseva… - Nephrology Dialysis …, 2016 - academic.oup.com
C3 glomerulopathy refers to a disease process in which abnormal control of complement
activation, degradation or deposition results in predominant C3 fragment deposition within …

Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea

V Brocklebank, D Kavanagh - Clinical kidney journal, 2017 - academic.oup.com
Thrombotic microangiopathy (TMA), characterized by organ injury occurring consequent to
severe endothelial damage, can manifest in a diverse range of diseases. In complement …