Explanipedia

Clinicopathological Spectrum and Treatment Outcomes of Cryofibrinogen-Associated Nephropathies Open

Julien Dang, Stanislas Faguer, Noémie Jourde‐Chiche, Vincent Javaugue, Frank Bridoux , et al. · 2025

C3 Glomerulonephritis Associated With Unusual IgG4 Antifactor H in IgG4-related Disease Open

Paul Dalmas, Mickaël Bobot, Noémie Jourde‐Chiche, Julie Bruno, Stéphane Burtey , et al. · 2025

A mouse model of cardiac immunoglobulin light chain amyloidosis reveals insights into tissue accumulation and toxicity of amyloid fibrils Open

Gemma Martinez-Rivas, Maria Victoria Ayala, Sébastien Bender, Gilles Roussine Codo, Weronika Karolina Swiderska , et al. · 2025

Using Electronic Patient-Reported Outcome Measures for Patients With Metastatic Renal Cell Carcinoma: A Narrative Review Open

Lauriane Minot, Vincent Javaugue, Marlène Arbutina, Sheik Emambux · 2025

ePROMs can be adapted to the toxicity and symptom specificities encountered in patients with mRCC undergoing front-line treatment. Combined with multidimensional HRQOL indicators, these tools can improve symptom management and provide earl…

A mouse model of cardiac AL amyloidosis unveils mechanisms of tissue accumulation and toxicity of amyloid fibrils Open

Gemma Martinez-Rivas, MV Ayala, Sébastien Bender, GR Codo, K Swiderska , et al. · 2024

AL amyloidosis is one of the most common types of systemic amyloidosis, caused by the deposition in tissues of fibrillar aggregates of abnormal immunoglobulin (Ig) light chain (LC), leading to organ dysfunction. The most frequent and sever…

Monoclonal Immunoglobulin Crystalline Membranous Nephropathy Open

Salvatore Mignano, Virginie Pascal, Nnaemezie E. Odioemene, William Forehand, Vincent Javaugue , et al. · 2024

Teclistamab in relapsed or refractory AL amyloidosis: a multinational retrospective case series Open

Nathalie Forgeard, Dikélélé Elessa, Alexander Carpinteiro, Karim Belhadj, Monique C. Minnema , et al. · 2023

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Table of Contents Open

Justin Cordova, Valentine Gillion, Karin Dahan, Cristina Dragean, Nathalie Demoulin , et al. · 2023

Light Chain–Only Immunotactoid Glomerulopathy: A Case Report Open

Lihong Bu, Vincent Javaugue, Sophie Chauvet, Jerold Napier, Surendra Dasari , et al. · 2022

The monotypic variant of immunotactoid glomerulopathy (ITG), strongly associated with low-grade lymphoproliferative disorders, is characterized histologically by glomerulonephritis and microtubular deposits of monoclonal immunoglobulin G (…

Nutritional biomarkers and heart failure requiring hospitalization in patients with type 2 diabetes: the SURDIAGENE cohort Open

Matthieu Wargny, Mikaël Croyal, Stéphanie Ragot, Elise Gand, David Jacobi , et al. · 2022

Atypical Antiglomerular Basement Membrane Nephritis Following Immune Checkpoint Inhibitor Open

Vincent Javaugue, Myra J. Watson, Fernando C. Fervenza, Samih H. Nasr · 2022

IntroductionAntiglomerular basement membrane (GBM) disease is caused by the presence of anti-GBM antibodies, usually directed against the noncollagenous domain of α-3 chain of type IV collagen (α3NC1), also called the Goodpasture antigen. …

Crystalglobulin-Associated Kidney Disease: A Case Report and Literature Review Open

Sophie Leflot, Philippe Leroy, Nathalie Demoulin, Selda Aydın, Guy Touchard , et al. · 2022

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…

Diagnosis and risk factors for intracranial aneurysms in autosomal polycystic kidney disease: a cross-sectional study from the Genkyst cohort Open

Siriane Lefèvre, Marie‐Pierre Audrézet, Jean‐Michel Halimi, Hélène Longuet, Frank Bridoux , et al. · 2022

Background Autosomal dominant polycystic kidney disease (ADPKD) is associated with an increased risk for developing intracranial aneurysms (IAs). We aimed to evaluate the frequency of diagnosis of IAs in the cross-sectional, population-bas…

Randall-Type Monoclonal Immunoglobulin Deposition Disease: New Insights into the Pathogenesis, Diagnosis and Management Open

Camille Cohen, Florent Joly, Audrey Sibille, Vincent Javaugue, Éstelle Desport , et al. · 2021

Randall-type monoclonal immunoglobulin deposition disease (MIDD) is a rare disease that belongs to the spectrum of monoclonal gammopathy of renal significance (MGRS). Renal involvement is prominent in MIDD, but extra-renal manifestations c…

Multiple Myeloma: An Overview of the Current and Novel Therapeutic Approaches in 2020 Open

Arthur Bobin, Évelyne Liuu, Niels Moya, Cécile Gruchet, Florence Sabirou , et al. · 2020

The survival rate of multiple myeloma (MM) patients has drastically increased recently as a result of the wide treatment options now available. Younger patients truly benefit from these innovations as they can support more intensive treatm…

Immunoglobulin light chain toxicity in a mouse model of monoclonal immunoglobulin light-chain deposition disease Open

Sébastien Bender, Maria Victoria Ayala, Amélie Bonaud, Vincent Javaugue, Claire Carrion , et al. · 2020

Light chain deposition disease (LCDD) is a rare disorder characterized by glomerular and peritubular amorphous deposits of a monoclonal immunoglobulin (Ig) light chain (LC), leading to nodular glomerulosclerosis and nephrotic syndrome. We …

New clinical forms of hereditary apoA-I amyloidosis entail both glomerular and retinal amyloidosis Open

Magali Colombat, Jean‐Claude Aldigier, Pierre-Raphaël Rothschild, Vincent Javaugue, Éstelle Desport , et al. · 2020

Immunoglobulin variable domain high-throughput sequencing reveals specific novel mutational patterns in POEMS syndrome Open

Sébastien Bender, Vincent Javaugue, Alexis Saintamand, Maria Victoria Ayala, Mehdi Alizadeh , et al. · 2020

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystem disease resulting from an underlying plasma cell (PC) dyscrasia. The pathophysiology of the disease remains unclea…

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

Barnabas Gellen, Nathalie Thorin‐Trescases, Éric Thorin, Elise Gand, P. Sosner , et al. · 2020

Monoclonal Gammopathy of Renal Significance Open

Vincent Javaugue, Céline Debiais-Delpech, Éstelle Desport, Laure Écotière, Jean‐Paul Fermand , et al. · 2020

Monoclonal gammopathy of renal significance. The term "monoclonal gammopathy of renal significance" regroups all renal disorders caused by a monoclonal immunoglobulin (Ig) secreted by a nonmalignant B-cell clone. However, a small clone can…

Full-length immunoglobulin high-throughput sequencing reveals specific novel mutational patterns in POEMS syndrome Open

Sébastien Bender, Vincent Javaugue, Alexis Saintamand, Maria Victoria Ayala, Mehdi Alizadeh , et al. · 2019

POEMS syndrome is a rare multisystem disease due to an underlying plasma cell (PC) dyscrasia. The pathophysiology of the disease remains unclear but the role of the monoclonal immunoglobulin (Ig) light chain (LC) is strongly suspected, due…

PS1406 WEEKLY CARFILZOMIB, LENALIDOMIDE AND DEXAMETHASONE UNTIL PROGRESSION IN RELAPSED REFRACTORY MULTIPLE MYELOMA Open

Cécile Gruchet, Valentine Richez, Stéphanie Guidez, Cécile Tomowiak, Guillemette Fouquet , et al. · 2019

Background: Carfilzomib, Lenalidomide and Dexamethasone association (KRd) has led to approval in early RRMM. On ASPIRE International phase 3 study Carfilzomib was used on a twice a week basis at 27 mg/m 2 and limited to 18 months exposure.…

PS1412 POMALIDOMIDE 3RD LINE VERSUS 4TH LINE FOR IN EARLY RELAPSED REFRACTORY MULTIPLE MYELOMA Open

Cécile Gruchet, Stéphanie Guidez, Cécile Tomowiak, Guillemette Fouquet, Antoine Machet , et al. · 2019

Background: Pomalidomide in association with dexamethasone is approved for relapsed and refractory Mutiple Myeloma (RRMM) in 3 rd line and beyond based on the multicenter international phase 3MM‐003 study that demonstrated greater efficacy…

Glomerulosclerosis and kidney failure in a mouse model of monoclonal immunoglobulin light-chain deposition disease Open

Sébastien Bender, Maria Victoria Ayala, Amélie Bonaud, Vincent Javaugue, Claire Carrion , et al. · 2019

Light chain deposition disease (LCDD) is a rare disorder characterized by glomerular and peritubular amorphous deposits of a monoclonal immunoglobulin (Ig) light chain (LC), leading to nodular glomerulosclerosis and nephrotic syndrome. We …

Randall-type monoclonal immunoglobulin deposition disease: novel insights from a nationwide cohort study Open

Florent Joly, Camille Cohen, Vincent Javaugue, Sébastien Bender, Mohamed Belmouaz , et al. · 2018

Monoclonal immunoglobulin deposition disease (MIDD) is a rare complication of B-cell clonal disorders, defined by Congo red negative–deposits of monoclonal light chain (LCDD), heavy chain (HCDD), or both (LHCDD). MIDD is a systemic disorde…

Comprehensive molecular characterization of a heavy chain deposition disease case Open

Sébastien Bender, Maria Victoria Ayala, Vincent Javaugue, Amélie Bonaud, Michel Cogné , et al. · 2018

International audience

Analysis of a Compartmental Model of Endogenous Immunoglobulin G Metabolism with Application to Multiple Myeloma Open

Felicity Kendrick, Neil D. Evans, Bertrand Arnulf, Hervé Avet‐Loiseau, Olivier Decaux , et al. · 2017

Immunoglobulin G (IgG) metabolism has received much attention in the literature for two reasons: (i) IgG homeostasis is regulated by the neonatal Fc receptor (FcRn), by a pH-dependent and saturable recycling process, which presents an inte…

Treatment of B-cell disorder improves renal outcome of patients with monoclonal gammopathy–associated C3 glomerulopathy Open

Sophie Chauvet, Véronique Frémeaux-Bacchi, Florent Petitprez, Alexandre Karras, Laurent Daniel , et al. · 2017

Key Points Monoclonal gammopathy is associated with C3 glomerulopathy. Specific treatment of the underlying B-cell clone improves renal survival.

SP121RANDALL-TYPE MIDD: THE DISEASE SPECTRUM Open

Florent Joly, Camille Cohen, Vincent Javaugue, Bertrand Arnulf, Bertrand Knebelmann , et al. · 2016

Prognostic value of kidney biopsy in myeloma cast nephropathy: a retrospective study of 70 patients Open

Laure Écotière, Antoine Thierry, Céline Debiais-Delpech, Sylvie Chevret, Vincent Javaugue , et al. · 2016

In the published version of this paper the first and second sentences of the 'Patients' section ('This study was done in accordance with the Declaration of Helsinki.Written informed consent was obtained from all study participants.Seventy …

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