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 …

[HTML][HTML] Current understanding of the role of complement in IgA nephropathy

N Maillard, RJ Wyatt, BA Julian, K Kiryluk… - Journal of the …, 2015 - ncbi.nlm.nih.gov
Complement activation has a role in the pathogenesis of IgA nephropathy, an autoimmune
disease mediated by pathogenic immune complexes consisting of galactose-deficient IgA1 …

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 …

[HTML][HTML] Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies

A Servais, LH Noël, LT Roumenina, M Le Quintrec… - Kidney international, 2012 - Elsevier
Dense deposit disease and glomerulonephritis with isolated C3 deposits are
glomerulopathies characterized by deposits of C3 within or along the glomerular basement …

Membranoproliferative glomerulonephritis—a new look at an old entity

S Sethi, FC Fervenza - New England Journal of Medicine, 2012 - Mass Medical Soc
This review discusses the causes, pathogenesis, and clinical management of
membranoproliferative glomerulonephritis, which accounts for 7 to 10% of biopsy-confirmed …

[HTML][HTML] Mayo clinic/renal pathology society consensus report on pathologic classification, diagnosis, and reporting of GN

S Sethi, M Haas, GS Markowitz, VD D'Agati… - Journal of the …, 2016 - ncbi.nlm.nih.gov
Renal pathologists and nephrologists met on February 20, 2015 to establish an
etiology/pathogenesis-based system for classification and diagnosis of GN, with a major aim …

[HTML][HTML] Eculizumab for dense deposit disease and C3 glomerulonephritis

AS Bomback, RJ Smith, GR Barile… - Clinical journal of the …, 2012 - ncbi.nlm.nih.gov
Results The subjects included three patients with dense deposit disease (including one
patient with recurrent dense deposit disease in allograft) and three patients with C3 …

[HTML][HTML] C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up

S Sethi, FC Fervenza, Y Zhang, L Zand, JA Vrana… - Kidney international, 2012 - Elsevier
C3 glomerulonephritis (C3GN) is a recently described disorder that typically results from
abnormalities in the alternative pathway (AP) of complement. Here, we describe the clinical …

[HTML][HTML] The complexity and heterogeneity of monoclonal immunoglobulin–associated renal diseases

S Sethi, SV Rajkumar, VD D'Agati - Journal of the American …, 2018 - ncbi.nlm.nih.gov
Monoclonal gammopathies are characterized by the overproduction of monoclonal Ig (MIg)
detectable in the serum or urine resulting from a clonal proliferation of plasma cells or B …

C3 glomerulopathy: a new classification

F Fakhouri, V Frémeaux-Bacchi, LH Noël… - Nature Reviews …, 2010 - nature.com
Several distinct pathological patterns of glomerular inflammation are associated with
abnormal regulation of the complement system, specifically, with dysregulation of the …