Update on C3 glomerulopathy: a complement-mediated disease

F Caravaca-Fontán, L Lucientes, T Cavero, M Praga - Nephron, 2020 - karger.com
Abstract C3 glomerulopathy (C3G) is a clinicopathologic entity secondary to dysregulation of
the alternative complement pathway in plasma and the glomerular microenvironment. The …

Mycophenolate mofetil in C3 glomerulopathy and pathogenic drivers of the disease

F Caravaca-Fontán… - Clinical Journal of the …, 2020 - journals.lww.com
Results The study group comprised 97 patients (84% C3 glomerulopathy, 16% dense
deposit disease). Forty-two patients were treated with corticosteroids plus MMF, and this …

Low‐molecular weight inhibitors of the alternative complement pathway

A Schubart, S Flohr, T Junt, J Eder - Immunological Reviews, 2023 - Wiley Online Library
Dysregulation of the alternative complement pathway predisposes individuals to a number
of diseases. It can either be evoked by genetic alterations in or by stabilizing antibodies to …

Complement and platelets: prothrombotic cell activation requires membrane attack complex–induced release of danger signals

M Mannes, V Pechtl, S Hafner, A Dopler… - Blood …, 2023 - ashpublications.org
Complement activation in the diseases paroxysmal nocturnal hemoglobinuria (PNH) and
atypical hemolytic uremic syndrome (aHUS) results in cytolysis and fatal thrombotic events …

Association of histologic parameters with outcome in C3 glomerulopathy and idiopathic immunoglobulin-associated membranoproliferative glomerulonephritis

HJ Lomax-Browne, NR Medjeral-Thomas… - Clinical Journal of the …, 2022 - journals.lww.com
Results Frequent biopsy features were mesangial expansion and hypercellularity,
glomerular basement membrane double contours, and endocapillary hypercellularity …

Clinical outcomes of patients with C3G or IC-MPGN treated with the factor D inhibitor danicopan: final results from two phase 2 studies

C Nester, GB Appel, AS Bomback… - American journal of …, 2022 - karger.com
Abstract Introduction: C3 glomerulopathy (C3G) is an ultrarare, chronic and progressive
nephropathy mediated by dysregulation of the alternative pathway of complement (AP), with …

[HTML][HTML] The glomerular crescent: triggers, evolution, resolution, and implications for therapy

L Anguiano, R Kain, HJ Anders - Current Opinion in Nephrology …, 2020 - journals.lww.com
The glomerular crescent: triggers, evolution, resolution, an... : Current Opinion in Nephrology
and Hypertension The glomerular crescent: triggers, evolution, resolution, and implications for …

CFHR gene variations provide insights in the pathogenesis of the kidney diseases atypical hemolytic uremic syndrome and C3 glomerulopathy

PF Zipfel, T Wiech, ED Stea… - Journal of the American …, 2020 - journals.lww.com
Sequence and copy number variations in the human CFHR–Factor H gene cluster
comprising the complement genes CFHR1, CFHR2, CFHR3, CFHR4, CFHR5, and Factor H …

[HTML][HTML] 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 …

Inherited kidney complement diseases

M Lemaire, D Noone, AL Lapeyraque… - Clinical Journal of the …, 2021 - journals.lww.com
In the past 20 years, we have witnessed tremendous advances in our ability to diagnose and
treat genetic diseases of the kidney caused by complement dysregulation. Staggering …