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2. Pathological characteristics of light chain crystalline podocytopathy

Author

Nasr, Samih H., Kudose, Satoru, Javaugue, Vincent, Harel, Stéphanie, Said, Samar M., Pascal, Virginie, Stokes, M. Barry, Vrana, Julie A., Dasari, Surendra, Theis, Jason D., Osuchukwu, George A., Sathick, Insara Jaffer, Das, Arjun, Kashkouli, Ali, Suchin, Elliot J., Liss, Yaakov, Suldan, Zalman, Verine, Jerome, Arnulf, Bertrand, Talbot, Alexis, Sethi, Sanjeev, Zaidan, Mohamad, Goujon, Jean-Michel, Valeri, Anthony M., Mcphail, Ellen D., Sirac, Christophe, Leung, Nelson, Bridoux, Frank, and D’Agati, Vivette D.

Published
2023
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5. Teclistamab in relapsed or refractory AL amyloidosis: a multinational retrospective case series

Author

Forgeard, Nathalie, primary, Elessa, Dikélélé, additional, Carpinteiro, Alexander, additional, Belhadj, Karim, additional, Minnema, Monique, additional, Roussel, Murielle, additional, Huart, Antoine, additional, Javaugue, Vincent, additional, Pascal, Laurent, additional, Royer, Bruno, additional, Talbot, Alexis, additional, Gounot, Romain, additional, Hegenbart, Ute, additional, Schonland, Stefan, additional, Karlin, Lionel, additional, Harel, Stéphanie, additional, Kastritis, Efstathios, additional, Bridoux, Frank, additional, Jaccard, Arnaud, additional, and Arnulf, Bertrand, additional

Published
2024
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7. New clinical forms of hereditary apoA-I amyloidosis entail both glomerular and retinal amyloidosis

Author

Colombat, Magali, Aldigier, Jean-Claude, Rothschild, Pierre-Raphael, Javaugue, Vincent, Desport, Estelle, Frouget, Thierry, Goujon, Jean-Michel, Rioux-Leclercq, Nathalie, Quellard, Nathalie, Rerolle, Jean Philippe, Paraf, François, Beugnet, Caroline, Tiple, Aurélien, Durrbach, Antoine, Samuel, Didier, Brézin, Antoine, Bridoux, Frank, and Valleix, Sophie

Published
2020
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8. Monoclonal Immunoglobulin Crystalline Membranous Nephropathy

Author

Mignano, Salvatore E., primary, Pascal, Virginie, additional, Odioemene, Nnaemezie E., additional, Forehand, William, additional, Javaugue, Vincent, additional, Said, Samar M., additional, Sethi, Sanjeev, additional, Sirac, Christophe, additional, and Nasr, Samih H., additional

Published
2024
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11. Treatment of B-cell disorder improves renal outcome of patients with monoclonal gammopathy–associated C3 glomerulopathy

Author

Chauvet, Sophie, Frémeaux-Bacchi, Véronique, Petitprez, Florent, Karras, Alexandre, Daniel, Laurent, Burtey, Stéphane, Choukroun, Gabriel, Delmas, Yahsou, Guerrot, Dominique, François, Arnaud, Le Quintrec, Moglie, Javaugue, Vincent, Ribes, David, Vrigneaud, Laurence, Arnulf, Bertrand, Goujon, Jean Michel, Ronco, Pierre, Touchard, Guy, and Bridoux, Frank

Published
2017
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16. Pathological characteristics of light chain crystalline podocytopathy

Author

Nasr, Samih H., primary, Kudose, Satoru, additional, Javaugue, Vincent, additional, Harel, Stéphanie, additional, Said, Samar M., additional, Pascal, Virginie, additional, Stokes, M. Barry, additional, Vrana, Julie A., additional, Dasari, Surendra, additional, Theis, Jason D., additional, Osuchukwu, George A., additional, Sathick, Insara Jaffer, additional, Das, Arjun, additional, Kashkouli, Ali, additional, Suchin, Elliot J., additional, Liss, Yaakov, additional, Suldan, Zalman, additional, Verine, Jerome, additional, Arnulf, Bertrand, additional, Talbot, Alexis, additional, Sethi, Sanjeev, additional, Zaidan, Mohamad, additional, Goujon, Jean-Michel, additional, Valeri, Anthony M., additional, Mcphail, Ellen D., additional, Sirac, Christophe, additional, Leung, Nelson, additional, Bridoux, Frank, additional, and D’Agati, Vivette D., additional

Published
2022
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20. Clinicopathologic and proteomic characteristics of intratubular cytoplasmic AL amyloidosis

Author

Javaugue, Vincent, primary, Rocha, Alejandro Best, additional, Said, Samar M., additional, Santoriello, Dominick, additional, Hou, Jean, additional, Dasari, Surendra, additional, Theis, Jason D., additional, Vrana, Julie A., additional, Batal, Ibrahim, additional, Larsen, Christopher, additional, Markowitz, Glen S., additional, D'Agati, Vivette D., additional, McPhail, Ellen D., additional, Leung, Nelson, additional, and Nasr, Samih H., additional

Published
2022
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22. Prognosis and long‐term outcomes in type I cryoglobulinemia: A multicenter study of 168 patients.

Author

Ghembaza, Amine, Boleto, Gonçalo, Bommelaer, Marin, Karras, Alexandre, Javaugue, Vincent, Bridoux, Frank, Alyanakian, Marie‐Alexandra, Molinier Frenkel, Valérie, Ghillani‐Dalbin, Pascale, Musset, Lucile, Barete, Stéphane, Roosweil, Damien, Choquet, Sylvain, Le Joncour, Alexandre, Mirouse, Adrien, Lipsker, Dan, Faguer, Stanislas, Vieira, Matheus, Cacoub, Patrice, and Biard, Lucie

Published
2023
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24. Crystalglobulin-Associated Kidney Disease: A Case Report and Literature Review.

Author

UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (SLuc) Centre du cancer, UCL - (SLuc) Service d'hématologie, UCL - (SLuc) Service de néphrologie, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - (SLuc) Service d'anatomie pathologique, Leflot, Sophie, Leroy, Philippe, Demoulin, Nathalie, Aydin, Selda, Touchard, Guy, Javaugue, Vincent, Vekemans, Marie-Christiane, Bridoux, Frank, Morelle, Johann, UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (SLuc) Centre du cancer, UCL - (SLuc) Service d'hématologie, UCL - (SLuc) Service de néphrologie, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - (SLuc) Service d'anatomie pathologique, Leflot, Sophie, Leroy, Philippe, Demoulin, Nathalie, Aydin, Selda, Touchard, Guy, Javaugue, Vincent, Vekemans, Marie-Christiane, Bridoux, Frank, and Morelle, Johann

Abstract

The kidney is commonly involved in multiple myeloma and other disorders producing monoclonal immunoglobulins. Crystalglobulinemia is a rare condition characterized by spontaneous crystallization and deposition of monoclonal immunoglobulins within the microvasculature of the kidney and other organs, leading to inflammation, ischemia, and end-organ damage. The present case and literature review highlight the clinical spectrum, diagnostic challenges, management, and outcomes of this underrecognized complication of monoclonal gammopathy. Crystalglobulin-associated kidney disease should be suspected in patients with rapidly progressive kidney disease associated with hematuria, proteinuria, extrarenal lesions (ie, skin and joints), and monoclonal gammopathy. Kidney biopsy is critical to the diagnosis, which relies on the identification by ultrastructural analysis of electron-dense crystalline structures composed of a monoclonal immunoglobulin within the kidney microvasculature. Conventional immunofluorescence on frozen tissue frequently fails to detect monoclonal protein deposits, and pronase-based antigen retrieval on paraffin-embedded material or immunoelectron microscopy is required to unmask antigenic epitopes located within crystalline inclusions. Early intervention combining treatment of clonal cell proliferation and plasma exchanges is warranted to reduce the burden of this rare but dramatic complication of monoclonal gammopathy.

Published
2022

28. Minimization of maintenance immunosuppressive therapy after renal transplantation comparing cyclosporine A/azathioprine or cyclosporine A/mycophenolate mofetil bitherapy to cyclosporine A monotherapy: a 10‐year postrandomization follow‐up study

Author

Thierry, Antoine, Lemeur, Yann, Ecotière, Laure, AbouAyache, Ramzi, Etienne, Isabelle, Laurent, Charlotte, Vuiblet, Vincent, Colosio, Charlotte, Bouvier, Nicolas, Aldigier, JeanClaude, Rerolle, JeanPhilippe, Javaugue, Vincent, Gand, Elise, Bridoux, Frank, Essig, Marie, Hurault de Ligny, Bruno, and Touchard, Guy

Published
2016
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29. Increased serum S100A12 levels are associated with higher risk of acute heart failure in patients with type 2 diabetes.

Author

Gellen, Barnabas, Thorin‐Trescases, Nathalie, Thorin, Eric, Gand, Elise, Ragot, Stephanie, Montaigne, David, Pucheu, Yann, Mohammedi, Kamel, Gatault, Philippe, Potier, Louis, Liuu, Evelyne, Hadjadj, Samy, Saulnier, Pierre‐Jean, Marechaud, Richard, Ragot, Stéphanie, Piguel, Xavier, Javaugue, Vincent, Hulin‐Delmotte, Charlotte, Llatty, Pierre, and Ducrocq, Gregory

Subjects
TYPE 2 diabetes, HEART failure patients, ADVANCED glycation end-products, BRAIN natriuretic factor, MAJOR adverse cardiovascular events
Abstract

Aims: The hyperglycaemic stress induces the release of inflammatory proteins such as S100A12, one of the endogenous ligands of the receptors for advanced glycation end products (RAGE). Chronic activation of RAGE has multiple deleterious effects in target tissues such as the heart and the vessels by promoting oxidative stress, inflammation by the release of cytokines, macrophages infiltration, and vascular cell migration and proliferation, causing ultimately endothelial cell and cardiomyocyte dysfunction. The aim of our study was to investigate the prognostic value of circulating S100A12 beyond established cardiovascular risk factors (CVRF) for heart failure (HF) and major adverse cardiovascular events (MACE) in a cohort of patients with type 2 diabetes. Methods and results: Serum S100A12 concentrations were measured at baseline in 1345 type 2 diabetes patients (58% men, 64 ± 11 years) recruited in the SURDIAGENE prospective cohort. Endpoints were the occurrence of acute HF requiring hospitalization (HHF) and MACE. We used a proportional hazard model adjusted for established CVRF (age, sex, duration of diabetes, estimated glomerular filtration rate, albumin/creatinine ratio, history of coronary artery disease) and serum S100A12. During the median follow‐up of 84 months, 210 (16%) and 505 (38%) patients developed HHF and MACE, respectively. Baseline serum S100A12 concentrations were associated with an increased risk of HHF [hazard ratio (HR) (95% confidence interval) 1.28 (1.01–1.62)], but not MACE [1.04 (0.90–1.20)]. After adjustment for CVRF, S100A12 concentrations remained significantly associated with an increased risk of HHF [1.29 (1.01–1.65)]. In a sub‐analysis, patients with high probability of pre‐existing HF [N terminal pro brain natriuretic peptide (NT‐proBNP) >1000 pg/mL, n = 87] were excluded. In the remaining 1258 patients, the association of serum S100A12 with the risk of HHF tended to be more pronounced [1.39 (1.06–1.83)]. When including the gold standard HF marker NT‐proBNP in the model, the prognostic value of S100A12 for HHF did not reach significance. Youden method performed at 7 years for HHF prediction yielded an optimal cut‐off for S100A12 concentration of 49 ng/mL (sensitivity 53.3, specificity 52.2). Compared with those with S100A12 ≤ 49 ng/mL, patients with S100A12 > 49 ng/mL had a significantly increased risk of HHF in the univariate model [HR = 1.58 (1.19–2.09), P = 0.0015] but also in the multivariate model [HR = 1.63 (1.23–2.16), P = 0.0008]. After addition of NT‐proBNP to the multivariate model, S100A12 > 49 ng/mL remained associated with an increased risk of HHF [HR = 1.42 (1.07–1.90), P = 0.0160]. However, the addition of S100A12 categories on top of multivariate model enriched by NT‐pro BNP did not improve the ability of the model to predict HHF (relative integrated discrimination improvement = 1.9%, P = 0.1500). Conclusions: In patients with type 2 diabetes, increased serum S100A12 concentration is independently associated with risk of HHF, but not with risk of MACE. Compared with NT‐proBNP, the potential clinical interest of S100A12 for the prediction of HF events remains limited. However, S100A12 could be a candidate for a multimarker approach for HF risk assessment in diabetic patients. [ABSTRACT FROM AUTHOR]

Published
2022
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30. Nutritional biomarkers and heart failure requiring hospitalization in patients with type 2 diabetes: the SURDIAGENE cohort.

Author

Wargny, Matthieu, Croyal, Mikaël, Ragot, Stéphanie, Gand, Elise, Jacobi, David, Trochu, Jean-Noël, Prieur, Xavier, Le May, Cédric, Goronflot, Thomas, Cariou, Bertrand, Saulnier, Pierre-Jean, Hadjadj, Samy, for the SURDIAGENE study group, Marechaud, Richard, Javaugue, Vincent, Hulin-Delmotte, Charlotte, Llatty, Pierre, Ducrocq, Gregory, Roussel, Ronan, and Rigalleau, Vincent

Subjects
HEART failure, TYPE 2 diabetes, LIQUID chromatography-mass spectrometry, TRIMETHYLAMINE, BIOMARKERS, CORONARY artery disease
Abstract

Background: Heart failure (HF) is a growing complication and one of the leading causes of mortality in people living with type 2 diabetes (T2D). Among the possible causes, the excess of red meat and the insufficiency of vegetables consumption are suspected. Such an alimentation is associated with nutritional biomarkers, including trimethylamine N-oxide (TMAO) and its precursors. Here, we aimed to study these biomarkers as potential prognostic factors for HF in patients with T2D. Methods: We used the SURDIAGENE (SURvival DIAbetes and GENEtics) study, a large, prospective, monocentric cohort study including 1468 patients with T2D between 2001 and 2012. TMAO and its precursors (trimethylamine [TMA], betaine, choline, and carnitine) as well as thio-amino-acids (cysteine, homocysteine and methionine) were measured by liquid chromatography-tandem mass spectrometry. The main outcome was HF requiring Hospitalization (HFrH) defined as the first occurrence of acute HF leading to hospitalization and/or death, established by an adjudication committee, based on hospital records until 31st December 2015. The secondary outcomes were the composite event HFrH and/or cardiovascular death and all-cause death. The association between the biomarkers and the outcomes was studied using cause-specific hazard-models, adjusted for age, sex, history of coronary artery disease, NT-proBNP, CKD-EPI-derived eGFR and the urine albumin/creatinine ratio. Hazard-ratios (HR) are expressed for one standard deviation. Results: The data of interest were available for 1349/1468 of SURDIAGENE participants (91.9%), including 569 (42.2%) women, with a mean age of 64.3 ± 10.7 years and a median follow-up of 7.3 years [25th–75th percentile, 4.7–10.8]. HFrH was reported in 209 patients (15.5%), HFrH and/or cardiovascular death in 341 (25.3%) and all-cause death in 447 (33.1%). In unadjusted hazard-models, carnitine (HR = 1.20, 95% CI [1.05; 1.37]), betaine (HR = 1.34, [1.20; 1.50]), choline (HR = 1.35, [1.20; 1.52]), TMAO (HR = 1.32, [1.16; 1.50]), cysteine (HR = 1.38, [1.21; 1.58]) and homocysteine (HR = 1.28, [1.17; 1.39]) were associated with HFrH, but not TMA and methionine. In the fully adjusted models, none of these associations was significant, neither for HFrH nor for HFrH and/or CV death, when homocysteine only was positively associated with all-cause death (HR = 1.16, [1.06; 1.27]). Conclusions: TMAO and its precursors do not appear to be substantial prognosis factors for HFrH, beyond usual cardiac- and kidney-related risk factors, whereas homocysteine is an independent risk factor for all-cause death in patients with T2D. [ABSTRACT FROM AUTHOR]

Published
2022
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32. Multiple Myeloma: An Overview of the Current and Novel Therapeutic Approaches in 2020

Author

Bobin, Arthur, primary, Liuu, Evelyne, additional, Moya, Niels, additional, Gruchet, Cécile, additional, Sabirou, Florence, additional, Lévy, Anthony, additional, Gardeney, Hélène, additional, Nsiala, Laly, additional, Cailly, Laura, additional, Guidez, Stéphanie, additional, Tomowiak, Cécile, additional, Systchenko, Thomas, additional, Javaugue, Vincent, additional, Durand, Géraldine, additional, Leleu, Xavier, additional, and Puyade, Mathieu, additional

Published
2020
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33. Immunoglobulin light chain toxicity in a mouse model of monoclonal immunoglobulin light-chain deposition disease

Author

Bender, Sébastien, primary, Ayala, Maria Victoria, primary, Bonaud, Amélie, primary, Javaugue, Vincent, primary, Carrion, Claire, primary, Oblet, Christelle, primary, Rinsant, Alexia, primary, Kaaki, Sihem, primary, Oruc, Zeliha, primary, Boyer, François, primary, Paquet, Agnes, primary, Pons, Nicolas, primary, Herve, Bastien, primary, Ashi, Mohamad Omar, primary, Jaccard, Arnaud, primary, Delpy, Laurent, primary, Touchard, Guy, primary, Cogne, Michel, primary, Bridoux, Frank, primary, and Sirac, Christophe, primary

Published
2020
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34. Immunoglobulin variable domain high-throughput sequencing reveals specific novel mutational patterns in POEMS syndrome

Author

Bender, Sébastien, primary, Javaugue, Vincent, primary, Saintamand, Alexis, primary, Ayala, Maria Victoria, primary, Alizadeh, Mehdi, primary, Filloux, Matthieu, primary, Pascal, Virginie, primary, Gachard, Nathalie, primary, Lavergne, David, primary, Auroy, Fabienne, primary, Cogné, Michel, primary, Bridoux, Frank, primary, Sirac, Christophe, primary, and Jaccard, Arnaud, primary

Published
2020
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35. Light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits is associated with a high detection rate of the pathogenic plasma cell clone

Author

Nasr, Samih H., primary, Larsen, Christopher P., additional, Sirac, Christophe, additional, Theis, Jason D., additional, Domenger, Camille, additional, Chauvet, Sophie, additional, Javaugue, Vincent, additional, Hogan, Jonathan J., additional, Said, Samar M., additional, Dasari, Surendra, additional, Vrana, Julie A., additional, McPhail, Ellen D., additional, Cornell, Lynn D., additional, Vilaine, Eve, additional, Massy, Ziad A., additional, Boffa, Jean-Jacques, additional, Buob, David, additional, Toussaint, Stéphanie, additional, Guincestre, Thomas, additional, Touchard, Guy, additional, D’Agati, Vivette D., additional, Leung, Nelson, additional, and Bridoux, Frank, additional

Published
2020
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37. Full-length immunoglobulin high-throughput sequencing reveals specific novel mutational patterns in POEMS syndrome

Author

Bender, Sébastien, primary, Javaugue, Vincent, additional, Saintamand, Alexis, additional, Ayala, Maria Victoria, additional, Alizadeh, Mehdi, additional, Filloux, Matthieu, additional, Pascal, Virginie, additional, Gachard, Nathalie, additional, Lavergne, David, additional, Auroy, Fabienne, additional, Cogne, Michel, additional, Bridoux, Frank, additional, Sirac, Christophe, additional, and Jaccard, Arnaud, additional

Published
2019
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39. Heavy Chain Fibrillary Glomerulonephritis: A Case Report

Author

Nasr, Samih H., primary, Sirac, Christophe, additional, Bridoux, Frank, additional, Javaugue, Vincent, additional, Bender, Sebastien, additional, Rinsant, Alexia, additional, Kaaki, Sihem, additional, Pinault, Emilie, additional, Dasari, Surendra, additional, Alexander, Mariam P., additional, Said, Samar M., additional, Hogan, Jonathan J., additional, Dispenzieri, Angela, additional, Touchard, Guy, additional, McPhail, Ellen D., additional, and Leung, Nelson, additional

Published
2019
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40. Clinicopathological spectrum of renal parenchymal involvement in B-cell lymphoproliferative disorders

Author

Javaugue, Vincent, primary, Debiais-Delpech, Céline, additional, Nouvier, Mathilde, additional, Gand, Elise, additional, Chauvet, Sophie, additional, Ecotiere, Laure, additional, Desport, Estelle, additional, Goujon, Jean-Michel, additional, Delwail, Vincent, additional, Guidez, Stéphanie, additional, Tomowiak, Cécile, additional, Leleu, Xavier, additional, Jaccard, Arnaud, additional, Rioux-Leclerc, Nathalie, additional, Vigneau, Cécile, additional, Fermand, Jean-Paul, additional, Touchard, Guy, additional, Thierry, Antoine, additional, and Bridoux, Frank, additional

Published
2019
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41. Weekly 20/56 mg/m² carfilzomib, lenalidomide, and dexamethasone until progression in early relapsed refractory multiple myeloma.

Author

Gruchet, Cecile, primary, Richez, Valentine, additional, Guidez, Stephanie, additional, Fouquet, Guillemette, additional, Azais, Isabelle, additional, Durand, Geraldine, additional, Javaugue, Vincent, additional, Plasse, Florent, additional, Machet, Antoine, additional, Moya, Niels, additional, Gardeney, Helene, additional, Bobin, Arthur, additional, Levy, Anthony, additional, Sabirou, Florence, additional, Bonnin, Anthony, additional, Dieval, Celine, additional, Primault, Sylvain, additional, Bridoux, Frank, additional, Avet-Loiseau, Herve, additional, and Leleu, Xavier, additional

Published
2019
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42. Glomerulosclerosis and kidney failure in a mouse model of monoclonal immunoglobulin light-chain deposition disease

Author

Bender, Sébastien, primary, Ayala, Maria Victoria, additional, Bonaud, Amélie, additional, Javaugue, Vincent, additional, Carrion, Claire, additional, Oblet, Christelle, additional, Rinsant, Alexia, additional, Quellard, Nathalie, additional, Kaaki, Sihem, additional, Oruc, Zeliha, additional, Boyer, François, additional, Paquet, Agnès, additional, Pons, Nicolas, additional, Hervé, Bastien, additional, Ashi, Mohamad Omar, additional, Jaccard, Arnaud, additional, Delpy, Laurent, additional, Touchard, Guy, additional, Cogné, Michel, additional, Bridoux, Frank, additional, and Sirac, Christophe, additional

Published
2019
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43. Carfilzomib Weekly 20/56mg/m², Lenalidomide and Dexamethasone for Early Relapsed Refractory Multiple Myeloma

Author

Richez, Valentine, primary, Gruchet, Cécile, additional, Guidez, Stephanie, additional, Fouquet, Guillemette, additional, Azais, Isabelle, additional, Durand, Geraldine, additional, Javaugue, Vincent, additional, Brigaud, Antoine, additional, Plasse, Florent, additional, Diolez, Jeremie, additional, Machet, Antoine, additional, Moya, Niels, additional, Gardeney, Helene, additional, Franques, Paul, additional, Bobin, Arthur, additional, Levy, Anthony, additional, Sabirou, Florence, additional, Bonnin, Anthony, additional, Dieval, Celine, additional, Primault, Sylvain, additional, Barrier, Jocelyn, additional, Fleck, Emmanuel, additional, Bouyier, Sabrina, additional, Daras, Claire, additional, Princet, Isabelle, additional, Bauwens, Delphine, additional, Legros, Laurence, additional, Fuzibet, Jean-Gabriel, additional, Gil, Angela, additional, Bridoux, Franck, additional, Avet-Loiseau, Hervé, additional, and Leleu, Xavier, additional

Published
2018
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44. Chronic HCV Infection and Autologous Stem Cell Transplantation for Multiple Myeloma

Author

Gruchet, Cécile, primary, Guidez, Stephanie, additional, Fouquet, Guillemette, additional, Azais, Isabelle, additional, Durand, Geraldine, additional, Javaugue, Vincent, additional, Brigaud, Antoine, additional, Plasse, Florent, additional, Diolez, Jeremie, additional, Sabirou, Florence, additional, Moya, Niels, additional, Bobin, Arthur, additional, Systchenko, Thomas, additional, Machet, Antoine, additional, Desmier, Deborah, additional, Levy, Anthony, additional, Dieval, Celine, additional, Primault, Sylvain, additional, Barrier, Jocelyn, additional, Corby, Anne, additional, Daras, Claire, additional, Princet, Isabelle, additional, Bauwens, Delphine, additional, Richez, Valentine, additional, Franques, Paul, additional, Gardeney, Helene, additional, Bouyer, Sabrina, additional, Dindinaud, Elodie, additional, Avet Loiseau, Herve, additional, Bridoux, Franck, additional, Christine, Silvain, additional, and Leleu, Xavier, additional

Published
2018
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45. Carfilzomib weekly 20/56 mg/m2 , lenalidomide and dexamethasone for early relapsed refractory multiple myeloma

Author

Richez, Valentine, primary, Gruchet, Cecile, additional, Guidez, Stephanie, additional, Fouquet, Guillemette, additional, Azais, Isabelle, additional, Durand, Geraldine, additional, Javaugue, Vincent, additional, Brigaud, Antoine, additional, Plasse, Florent, additional, Diolez, Jéremie, additional, Machet, Antoine, additional, Moya, Niels, additional, Gardeney, Helene, additional, Franques, Paul, additional, Bobin, Arthur, additional, Levy, Anthony, additional, Sabirou, Florence, additional, Bonnin, Anthony, additional, Dieval, Celine, additional, Primault, Sylvain, additional, Barrier, Jocelyn, additional, Fleck, Emmanuel, additional, Bouyier, Sabrina, additional, Daras, Claire, additional, Princet, Isabelle, additional, Bauwens, Delphine, additional, Legros, Laurence, additional, Fuzibet, Jean-Gabriel, additional, Gil, Angela, additional, Bridoux, Frank, additional, Avet-Loiseau, Hervé, additional, and Leleu, Xavier, additional

Published
2018
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46. Proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a nephrologist perspective.

Author

Bridoux, Frank, Javaugue, Vincent, Nasr, Samih H, and Leung, Nelson

Subjects
*IGA glomerulonephritis, *MONOCLONAL gammopathies, *DIAGNOSIS, *CHRONIC kidney failure, *IMMUNOGLOBULIN G, *KIDNEY physiology
Abstract

Proliferative glomerulonephritis (GN) with monoclonal immunoglobulin deposits (PGNMIDs) is a recently described entity among the spectrum of monoclonal gammopathy of renal significance (MGRS). The disease is renal limited and manifests with chronic glomerular disease, altered renal function and albuminuria, sometimes in the nephrotic range. Acute nephritic syndrome is rare. PGNMID occurs mostly in the sixth decade, but it may affect young adults. Histologically, PGNMID is characterized predominantly by membranoproliferative GN and less frequently by diffuse endocapillary GN, mesangioproliferative GN or atypical membranous GN. Immunofluorescence and electron microscopic studies are the cornerstone of diagnosis, showing granular deposits involving glomeruli only, and composed of monotypic immunoglobulin G (IgG), with a single heavy chain subclass (most commonly IgG3) and light chain (LC) restriction (usually κ), admixed with complement deposits. PGNMID variants with monotypic LC-only, IgA or IgM deposits are uncommon. Ultrastructurally, deposits are amorphous with predominant subendothelial and mesangial distribution. PGNMID should be distinguished from type 1 cryoglobulinemic GN and immunotactoid GN, which share some common pathological features. Contrary to other MGRS lesions, the rate of detection of the nephrotoxic monoclonal Ig in the serum or urine, and of an abnormal bone marrow B-cell clone, is only ∼30%. Renal prognosis is poor, with progression to end-stage renal disease in 25% of patients within 30 months and frequent early recurrence on the renal allograft. The pathophysiology of PGNMID is unclear and its treatment remains challenging. However, recent studies indicate that clone-targeted chemotherapy may significantly improve renal outcomes, opening future perspectives for the management of this rare disease. [ABSTRACT FROM AUTHOR]

Published
2021
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47. Serum tenascin-C is independently associated with increased major adverse cardiovascular events and death in individuals with type 2 diabetes: a French prospective cohort.

Author

Gellen, Barnabas, Thorin-Trescases, Nathalie, Thorin, Eric, Gand, Elise, Sosner, Philippe, Brishoual, Sonia, Rigalleau, Vincent, Montaigne, David, Javaugue, Vincent, Pucheu, Yann, Gatault, Philippe, Piguel, Xavier, Hadjadj, Samy, and Saulnier, Pierre-Jean

Abstract

Aims/hypothesis: Tenascin-C (TN-C) is an extracellular matrix glycoprotein highly expressed in inflammatory and cardiovascular (CV) diseases. Serum TN-C has not yet been specifically studied in individuals with type 2 diabetes, a condition associated with chronic low-grade inflammation and increased CV disease risk. In this study, we hypothesised that elevated serum TN-C at enrolment in participants with type 2 diabetes would be associated with increased risk of death and major adverse CV events (MACE) during follow-up. Methods: We used a prospective, monocentric cohort of consecutive type 2 diabetes participants (the SURDIAGENE [SUivi Rénal, DIAbète de type 2 et GENEtique] cohort) with all-cause death as a primary endpoint and MACE (CV death, non-fatal myocardial infarction or stroke) as a secondary endpoint. We used a proportional hazard model after adjustment for traditional risk factors and the relative integrated discrimination improvement (rIDI) to assess the incremental predictive value of TN-C for these risk factors. Results: We monitored 1321 individuals (58% men, mean age 64 ± 11 years) for a median of 89 months. During follow-up, 442 individuals died and 497 had MACE. Multivariate Cox analysis showed that serum TN-C concentrations were associated with an increased risk of death (HR per 1 SD: 1.27 [95% CI 1.17, 1.38]; p < 0.0001) and MACE (HR per 1 SD: 1.23 [95% CI 1.13, 1.34]; p < 0.0001). Using TN-C concentrations on top of traditional risk factors, prediction of the risk of all-cause death (rIDI: 8.2%; p = 0.0006) and MACE (rIDI: 6.7%; p = 0.0014) improved significantly, but modestly. Conclusions/interpretation: In individuals with type 2 diabetes, increased serum TN-C concentrations were independently associated with death and MACE. Therefore, including TN-C as a prognostic biomarker could improve risk stratification in these individuals. [ABSTRACT FROM AUTHOR]

Published
2020
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49. Animal models of monoclonal immunoglobulin-related renal diseases

Author

Sirac, Christophe, primary, Herrera, Guillermo A., additional, Sanders, Paul W., additional, Batuman, Vecihi, additional, Bender, Sebastien, additional, Ayala, Maria V., additional, Javaugue, Vincent, additional, Teng, Jiamin, additional, Turbat-Herrera, Elba A., additional, Cogné, Michel, additional, Touchard, Guy, additional, Leung, Nelson, additional, and Bridoux, Frank, additional

Published
2018
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50. Impaired Lysosomal Function Underlies Monoclonal Light Chain-Associated Renal Fanconi Syndrome

Author

Luciani, Alessandro, Sirac, Christophe, Terryn, Sara, Javaugue, Vincent, Prange, Jenny Ann, Bender, Sébastien, Bonaud, Amélie, Cogné, Michel, Aucouturier, Pierre, Ronco, Pierre, Bridoux, Frank, Devuyst, Olivier, University of Zurich, and Bridoux, Frank

Subjects
2727 Nephrology, 10076 Center for Integrative Human Physiology, 570 Life sciences, biology, 610 Medicine & health, 10052 Institute of Physiology
Published
2016

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