Validation of a Histologic Scoring Index for C3 Glomerulopathy

Caravaca-Fontán, Fernando; Trujillo, Hernando; Alonso, Marina; Díaz-Encarnación, Montserrat; Cabello, Virginia; Ariceta, Gema; Quintana, Luís F.; Marco, Helena; Barros, Xoana; Ramos, Natália; Rodríguez-Mendiola, Nuria; Cruz, Sonia; Fernández-Juárez, Gema; Rodríguez, Eva; Cerda, Francisco de la; José, Ana Pérez de; López, Inmaculada; Fernández, Loreto; Gómez, Vanessa Pérez; Ávila, Ana; Bravo, Luis Eduardo; Lumbreras, Javier; Allende, Natalia; Nieta, María Dolores Sánchez de la; Olea, Teresa; Melgosa, Marta; Huerta, Ana; Miquel, Rosa; Mon, Carmen; Fraga, Gloria; Lorenzo, Alberto de; Draibe, Juliana; González, Fayna; Shabaka, Amir; Illescas, María Luisa; Calvo, Consuelo; Oviedo, Victoria; Silva, Iara da; Jorge, Elena Goicoechea de; Caravaca, Francisco; Praga, Manuel; Cavero, Teresa; Sevillano, Ángel; Gutiérrez, Eduardo; Morales, Enrique; González, Lucía Gómez; Galán, Rafael Camacho; Domínguez, Alejandra Gabaldón; Garrido, M C; Navarro, Alexandra; Cortes, José A.; Martin, Alejando Pascual; Pérez-Ebri, M. Luisa; Cabrera‐Pérez, Rocío; Santos, Ángel Panizo; Gregorio, Laura Yébenes; Fernández, Maria Eugenia García; Gimeno, Javier; Ortiz, Pablo Cannata; Sastre, Esther; Sáiz, Ana; Ruiz, Eduardo Salido; Rodriguez, R. Ruiz; Cuevas, Cesáreo Corbacho; Crespo, Francisco Díaz; Arce, Yolanda; Calvar, Maria Soledad Garcia-Cuerva; Saus, Carles; Márquez, Carmen Guerrero; García‐Herrera, Adriana; Gallego, Montserrat Gomà; Álvarez, Dolores López; Muñoz, Cristina Meléndez; Centeno, Macarena; Ñíguez, Belén Ferri; Reboredo, Juan Mosquera; Martul, Eduardo Vázquez; Gutiérrez, Sofía Pérez

doi:10.1053/j.ajkd.2020.11.011

Cited by 27 publications

(25 citation statements)

References 23 publications

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“…Patients were considered to have dense deposit disease (DDD) when highly electron-dense intramembranous deposits were observed on electron microscopy, and C3 glomerulonephritis (C3GN) when deposits did not fulfill this criterion. Baseline and follow-up data were compiled from the medical records of all participating centers, as described elsewhere [ 4 , 7 ].…”

Section: Methodsmentioning

confidence: 99%

“…Kidney biopsy specimens were examined in the pathology departments of the participating hospitals. The degree of disease activity and chronicity was analyzed according to the previously published C3G histologic index [ 4 , 5 ], using a semiquantitative scale of 0–3, except for arteriosclerosis, which was scored as 0–1. Total activity and chronicity scores were then calculated as the sum of each individual item, with a total possible score of 21 for activity lesions and a total possible score of 10 for chronic lesions.…”

Section: Methodsmentioning

confidence: 99%

“…In recent years, several important efforts have led to a better understanding of the pathogenesis and natural history of C3G [ 1 , 3 ]. However, the often slowly progressive and pauci-symptomatic course of the disease at its early stages may lead to a delayed diagnosis of the disease in many cases, with the consequent development of irreversible chronic histologic lesions [ 3 , 4 ]. In fact, the extent of disease chronicity in kidney biopsy has been recognized as a major predictor of kidney outcomes in C3G [ 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Development and validation of a nomogram to predict kidney survival at baseline in patients with C3 glomerulopathy

Caravaca-Fontán

Rivero

Cavero

et al. 2022

Clinical Kidney Journal

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Background C3 glomerulopathy is a rare and heterogeneous complement-driven disease. It is often challenging to accurately predict in clinical practice the individual kidney prognosis at baseline. We herein sought to develop and validate a prognostic nomogram to predict the long-term kidney survival. Methods Retrospective, multicenter observational cohort study in 35 nephrology departments belonging to the GLOSEN group. The dataset was randomly divided into training group (n = 87) and validation group (n = 28). The least absolute shrinkage and selection operator (LASSO) regression was used to screen the main predictors of kidney outcome, and used to build the nomogram. Accuracy of the nomogram was assessed by discrimination and risk calibration, in the training and validation sets. Results The study group comprised 115 patients, of whom 46 patients (40%) reached kidney failure in a median follow-up of 49 months (range 24–112). No significant differences were observed in baseline estimated glomerular filtration rate (eGFR), proteinuria or total chronicity score of kidney biopsy, between patients in the training versus those in the validation set. The selected variables by LASSO were eGFR, proteinuria and total chronicity score. Based on Cox model a nomogram was developed for the prediction of kidney survival at 1-, 2-, 5- and 10-years from diagnosis. The C-index of the nomogram was 0.860 (95% confidence interval 0.834–0.887), and calibration plots showed optimal agreement between predicted and observed outcomes. Conclusions We constructed and validated a practical nomogram with good discrimination and calibration, to predict the risk of kidney failure in C3G patients at 1-, 2-, 5- and 10-years.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Development and validation of a nomogram to predict kidney survival at baseline in patients with C3 glomerulopathy

Caravaca-Fontán

Rivero

Cavero

et al. 2022

Clinical Kidney Journal

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“…Larger observational or prospective studies that include histologic descriptions should be performed in order to get a better understanding of the true epidemiology of ARN. [6,7] ‡ IgAN vs Non-IgAN Glomerulosclerosis (0-3) IFTA (0-6) Arterio-or arteriolosclerosis (absent vs present) Total Chronicity Score (0-10)…”

Section: J O U R N a L P R E -P R O O Fmentioning

confidence: 99%

IgA Nephropathy Is the Most Common Underlying Disease in Patients With Anticoagulant-Related Nephropathy

Trujillo

Pérez

Cavero

et al. 2022

Kidney International Reports

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“…In C3 glomerulopathy, the total chronicity score was determined as the principal histological predictive factor for KF [ 41 , 42 ]. In diabetic nephropathy, the severity of chronic changes, especially GS and IFTA, are related with renal outcomes [ 43 ]. The present study shows that the MCCS grade also stratifies patients with AAV-GN into groups with different profiles of eGFR, renal recovery and risk for renal outcomes.…”

Section: Discussionmentioning

confidence: 99%

Kidney biopsy chronicity grading in antineutrophil cytoplasmic antibody-associated vasculitis

Moura

Fervenza

Specks

et al. 2021

Nephrology Dialysis Transplantation

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Background Kidney biopsy is valuable for prognostic assessment of renal outcomes in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with glomerulonephritis (AAV-GN) but the impact of chronic changes is not determined. Methods We conducted a retrospective cohort study of myeloperoxidase (MPO)- or proteinase 3 (PR3)-ANCA-positive patients with AAV and active renal disease. We applied the Mayo Clinic Chronicity Score (MCCS) and validated and evaluated its implications on outcome prediction in AAV-GN. Results We analyzed 329 patients with kidney biopsies available to score. The extent of chronicity was graded by MCCS as minimal [102 (31.0%)], mild [106 (32.2%)], moderate [86 (26.1%)] and severe [35 (10.6%)]. The MCCS grades correlated with the degree of renal function impairment at presentation [mean estimated glomerular filtration rate (eGFR) 48.3 versus 29.2 versus 23.7 versus 18.5 mL/min/1.73 m2, respectively; P < 0.0001]. Higher degrees of the individual components of the MCCS (glomerulosclerosis, interstitial fibrosis, tubular atrophy and arteriosclerosis) were associated with lower median eGFR (P < 0.0001) and decreased event-free [kidney failure (KF) and death] survival (P = 0.002, P < 0.0001, P < 0.0001 and P = 0.017, respectively). Patients with lower MCCS grades recovered renal function more frequently (P < 0.0001). Increasing MCCS grades were associated with decreased renal recovery (P = 0.001), more frequent events and shorter time to KF (P < 0.0001), KF and death (P < 0.0001) and death (P = 0.042), independent of the remission induction treatment used (cyclophosphamide or rituximab). The MCCS stratified renal outcomes for each MCCS grade and can be used in clinical practice as a cutoff for KF prediction (MCCS ≥4). Conclusions Chronic changes on kidney histology independently predict renal function, outcomes and response to treatment in AAV-GN.

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Validation of a Histologic Scoring Index for C3 Glomerulopathy

Cited by 27 publications

References 23 publications

Development and validation of a nomogram to predict kidney survival at baseline in patients with C3 glomerulopathy

Development and validation of a nomogram to predict kidney survival at baseline in patients with C3 glomerulopathy

IgA Nephropathy Is the Most Common Underlying Disease in Patients With Anticoagulant-Related Nephropathy

Kidney biopsy chronicity grading in antineutrophil cytoplasmic antibody-associated vasculitis

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