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Your search keyword '"Etienne Riviere"' showing total 23 results
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23 results on '"Etienne Riviere"'

1. Efficacy and safety of two rituximab biosimilars for treating immune thrombocytopenia: a reference-product matched study

Author

Arthur Mageau, Bernard Bonnotte, Mikael Ebbo, Antoine Dossier, Lionel Galicier, Odile Souchaud-Debouverie, Corentin Orvain, Mathieu Gerfaud-Valentin, Delphine Gobert, Etienne Riviere, Sylvain Audia, Matthieu Mahevas, Marc Michel, Jean-Francois Viallard, and Bertrand Godeau

Subjects
biosimilar, immune thrombocytopenia, itp, rituximab, Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

The emergence of rituximab biosimilars offers the prospect of significant savings to the healthcare system. However, these drugs have never been evaluated for treating immune thrombocytopenia (ITP). This was an observational, matched study. We included adults who received a rituximab biosimilar for ITP. Each rituximab-naïve biosimilar patient was matched with two controls from the historic ITP-ritux registry. For non-naïve patients, we compared the response to the biosimilar with that observed with the reference product. Response status was defined according to international criteria. We included 107 patients; 55 receiving Rixathon™ and 52 Truxima™. Three months after the first infusion of rituximab biosimilars, the overall response rate was 47/74 (63.5%) versus 76/142 (53.5%) for the matched controls receiving the reference product (p = .13). The 3-month overall response rate was 76.5% for Rixathon™ versus 51.5% for the matched control group (p = .01) and 21/40 (52.5%) for Truxima™ versus 41/74 (55.4%) for the matched controls (p = .81). For non-naïve patients, the response pattern was similar to that observed previously with the reference product. Safety was analogous to that observed with the reference product. Rituximab biosimilars seemed safe and effective for ITP treatment.

Published
2023
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2. Insulin amyloidosis: A case report

Author

Arthur Dubernet, Maud Toulmonde, Magali Colombat, Cécile Hartog, and Etienne Riviere

Subjects
amyloidosis, insulin, type 1 diabetes, hyperglycemia, case report, Medicine (General), R5-920
Abstract

Insulin amyloidosis is a rare form of localized amyloidosis due to insulin aggregation into subcutaneous amyloid fibrils. We describe the case of a 55 years old male with insulin-requiring type 1 diabetes presenting with two non-inflammatory intra-dermal nodules associated with local lymph node enlargement. Diagnosis was confirmed by Congo red coloration of the amyloid deposit and insulin protein identification on mass spectrometry. Insulin amyloidosis is a potential complication of repeated subcutaneous insulin injections. The main risk factor is the intrinsic characteristic of the insulin used. Insulin amyloidosis leads to systemic metabolic consequences such as chronic hyperglycemia or unpredictable hypoglycemia, as well as unesthetic cutaneous lumps or abscesses. Standard-of-care is yet to be defined but mainly rely on therapeutical education of insulin injections, while surgical excision is reported to improve glycemic control in some patients.

Published
2023
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3. Impact of Mutational Status and Prognostic Factors on Survival in Chronic Myelomonocytic Leukemia With Systemic Inflammation and Autoimmune Disorders

Author

Charles Dussiau, Henry Dupuy, Audrey Bidet, Mathieu Sauvezie, Anne-Charlotte De-Grande, Lisa Boureau, Etienne Riviere, Edouard Forcade, Fabrice Bonnet, Pierre-Yves Dumas, Pierre Duffau, Arnaud Pigneux, Jean-François Viallard, Sophie Dimicoli-Salazar, and Estibaliz Lazaro

Subjects
Diseases of the blood and blood-forming organs, RC633-647.5
Published
2023
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4. Prolonged response after TPO-RA discontinuation in primary ITP: results of a prospective multicenter study

Author

Stephanie Guillet, Etienne Crickx, Imane Azzaoui, pascal chappert, Emmanuelle Boutin, Jean-François Viallard, Etienne Riviere, Delphine Gobert, Lionel Galicier, Marion Malphettes, Stéphane Cheze, Francois Lefrere, Sylvain Audia, Bernard Bonnotte, Olivier Lambotte, Nicolas Noel, Olivier Fain, Guillaume Moulis, Mohamed Hamidou, Mathieu Gerfaud-Valentin, Jean-Pierre Marolleau, Louis Terriou, Nihal Martis, Anne-Sophie Morin, Antoinette Perlat, Thomas Le Gallou, Frédérique Roy-Peaud, Ailsa Robbins, Jean-Christophe Lega, Mathieu Puyade, Thibault Comont, Nicolas Limal, Laetitia Languille, Anissa Zarour, Marine Luka, Mickaël Mathieu Ménager, Thibaut Belmondo, Sophie Hue, Florence Canoui-Poitrine, Marc Michel, Bertrand Godeau, Matthieu Mahevas, Service de médecine interne [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Université d'Orléans (UO)-Centre National de la Recherche Scientifique (CNRS), CHU Henri Mondor [Créteil], Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), IMRB - CEPIA/'Clinical Epidemiology And Ageing : Geriatrics, Primary Care and Public Health' [Créteil] (U955 Inserm - UPEC), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Université de Bordeaux (UB), CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Necker - Enfants Malades [AP-HP], Service de médecine interne et immunologie clinique (SOC 1) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Immunologie des Maladies Virales et Autoimmunes (IMVA - U1184), Université Paris-Sud - Paris 11 (UP11)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM), AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Service de Médecine Interne - Immunologie Clinique [AP-HP Bicêtre], Infectious Diseases Models for Innovative Therapies (IDMIT), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay, Centre d'investigation clinique de Toulouse (CIC 1436), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Pôle Santé publique et médecine publique [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre de Recherche en Transplantation et Immunologie - Center for Research in Transplantation and Translational Immunology (U1064 Inserm - CR2TI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Nantes Université - UFR de Médecine et des Techniques Médicales (Nantes Univ - UFR MEDECINE), Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ), Institut de transplantation urologie-néphrologie (ITUN), Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes), Hospices Civils de Lyon (HCL), CHU Amiens-Picardie, HEMATIM - Hématopoïèse et immunologie - UR UPJV 4666 (HEMATIM), and Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Immunology, Cell Biology, Hematology, Biochemistry, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

Sustained response off-treatment (SROT) after thrombopoietin receptor agonist (TPO-RA) discontinuation has been reported in ITP. This prospective multicenter interventional study enrolled adults with persistent or chronic primary ITP and complete response on TPO-RAs. The primary endpoint was the proportion of patients achieving SROT (platelet count > 30x109/L and no bleeding) at W24 with no other ITP-specific medications. Secondary endpoints included the proportion of sustained complete response off-treatment (SCROT, platelet count > 100x109/L and no bleeding) and SROT at W52, bleeding events, and pattern of response to a new course of TPO-RAs. We included 48 patients with median (IQR) age 58.5 years (41-73.5); 30/48 (63%) had chronic ITP at TPO-RA initiation. In the intention-to-treat analysis, 27/48 (56.2%, 95% CI, 41.2-70.5) achieved SROT; 15/48 (31.3%; 95% CI, 18.9-44.5) achieved SCROT at W24, and 25/48 (52.1%; 95% CI, 37.2-66.7) achieved respectively SROT and 14/48 (29.2%; 95% CI, 17.2-42.3) SCROT at W52. No severe bleeding episode occurred in patients who relapsed. Among patients re-challenged with TPO-RA, 11/12 achieved CR. We found no significant clinical predictors of SROT at W24. Single-cell RNA-seq revealed enrichment of a "TNFα signaling via NF-κB" signature in CD8+ T cells of patients with no sustained response after TPO-RA discontinuation, which was further confirmed by a significant overexpression of CD69 on CD8+ T cells at baseline in these patients as compared with those achieving SCROT/SROT. Our results strongly support a strategy based of progressive tapering and discontinuation of TPO-RAs for patients with chronic ITP who achieved a stable CR on treatment. Clinical trial number: NCT03119974

Published
2023
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5. Characteristics and outcome of adults with severe autoimmune hemolytic anemia admitted to the intensive care unit: Results from a large French observational study

Author

Clara Pouchelon, Charlotte Lafont, Antoine Lafarge, Thibault Comont, Etienne Riviere, David Boutboul, Claire Fieschi, Antoine Dossier, Sylvain Audia, Julien Vaidie, Marc Ruivard, Delphine Gobert, Guillaume Bonnard, Julie Graveleau, Matthieu Mahevas, Bertrand Godeau, and Marc Michel

Subjects
Adult, Hospitalization, Anemia, Hemolytic, Intensive Care Units, Humans, Hematology, Anemia, Hemolytic, Autoimmune
Published
2022

6. The 2024 French guidelines for scenario design in simulation-based education: manikin-based immersive simulation, simulated participant-based immersive simulation and procedural simulation

Author

Guillaume Der Sahakian, Maxime de Varenne, Clément Buléon, Guillaume Alinier, Christian Balmer, Antonia Blanié, Bertrand Bech, Anne Bellot, Hamdi Boubaker, Nadège Dubois, Francisco Guevara, Erwan Guillouet, Jean-Claude Granry, Morgan Jaffrelot, François Lecomte, Fernande Lois, Mohammed Mouhaoui, Ollivier Ortolé, Méryl Paquay, Justine Piazza, Marie Pittaco, Patrick Plaisance, Dan Benhamou, Gilles Chiniara, and Etienne Rivière

Subjects
Simulation-based education, scenario design, simulated patient, procedural simulation, immersive simulation, crisis resource management, Special aspects of education, LC8-6691, Medicine (General), R5-920
Abstract

Background Simulation-based education in healthcare encompasses a wide array of modalities aimed at providing realistic clinical experiences supported by meticulously designed scenarios. The French-speaking Society for Simulation in Healthcare (SoFraSimS) has developed guidelines to assist educators in the design of scenarios for manikin- or simulated participant- based immersive simulation and procedural simulation, the three mainly used modalities.Methods After establishing a French-speaking group of experts within the SoFraSimS network, we performed an extensive literature review with theory-informed practices and personal experiences. We used this approach identify the essential criteria for practice-based scenario design within the three simulation modalities.Results We present three comprehensive templates for creating innovative scenarios and simulation sessions, each tailored to the specific characteristics of a simulation modality. The SoFraSimS templates include five sections distributed between the three modalities. The first section contextualizes the scenario by describing the practicalities of the setting, the instructors and learners, and its connection to the educational program. The second section outlines the learning objectives. The third lists all the elements necessary during the preparation phase, describing the educational method used for procedural simulation (such as demonstration, discovery, mastery learning, and deliberate practice). The fourth section addresses the simulation phase, detailing the behaviors the instructor aims to analyze, the embedded triggers, and the anticipated impact on simulation proceedings (natural feedback). This ensures maximum control over the learning experience. Finally, the fifth section compiles elements for post-simulation modifications to enhance future iterations.Conclusion We trust that these guidelines will prove valuable to educators seeking to implement simulation-based education and contribute to the standardization of scenarios for healthcare students and professionals. This standardization aims to facilitate communication, comparison of practices and collaboration across different learning and healthcare institutions.

Published
2024
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7. C2B2: a Cloud-native Chaos Benchmarking suite for the Hyperledger Fabric Blockchain

Author

Ayham Kassab, Etienne Riviere, Guillaume Rosinosky, Ramin Sadre, Viet Hoang Tran, and UCL - SST/ICTM/INGI - Pôle en ingénierie informatique

Abstract

We present C2B2, an integrated suite for chaos engineering, benchmarking, and capacity planning of large-scale, multi-cloud Hyperledger Fabric installations. C2B2 is cloud-native and adopts an everything-as-code approach, facilitating the reproducibility of experiments by streamlining their deployment on Kubernetes, while offering simplified and flexible configuration to developers. C2B2 enables large-scale testing of complex multi-cloud deployment scenarios through network emulation, and its chaos engineering features allow simultaneously evaluating the impact of faults or network instabilities. Our evaluation of C2B2 demonstrates its interest in terms of the reduction of the experiment setup complexity for two representative scenarios, the evaluation of the impact of a consortium organic growth, and the evaluation of the impact of geo-distribution on performance.

Published
2022

8. Absence of severe COVID-19 in patients with clonal mast cells activation disorders: effective anti-SARS-CoV-2 immune response

Author

Julien Rossignol, Amani Ouedrani, Cristina Bulai Livideanu, Stéphane Barete, Louis Terriou, David Launay, Richard Lemal, Celine Greco, Laurent Frenzel, Cecile Meni, Christine Bodemere-Skandalis, Laura Polivka, Anne-Florence Collange, Hassiba Hachichi, Sonia Bouzourine, Djazira Nait Messaoud, Mathilde Negretto, Laurence Vendrame, Marguerite Jambou, Marie Gousseff, Stéphane Durupt, Jean-Christophe Lega, Jean-Marc Durand, Caroline Gaudy, Gandhi Damaj, Marie-Pierre Gourin, Mohamed Hamidou, Laurence Bouillet, Edwige Le Mouel, Alexandre Maria, Patricia Zunic, Quentin Cabrera, Denis Vincent, Christian Lavigne, Etienne Riviere, Clement Gourguechon, Anne Brignier, Ludovic Lhermitte, Thierry Jo Molina, Julie Bruneau, Julie Agopian, Patrice Dubreuil, Dana Ranta, Alexandre Mania, Michel Arock, Isabelle Staropoli, Olivier Tournilhac, Olivier Lortholary, Olivier Schwartz, Lucienne Chatenoud, and Olivier Hermine

Subjects
education.field_of_study, Innate immune system, biology, business.industry, ELISPOT, Population, Tryptase, Acquired immune system, Immune system, Immunity, Immunology, biology.protein, Medicine, Antibody, education, business
Abstract

Mast cells are key actors of innate immunity and Th2 adaptive immune response which counterbalance Th1 response, critical for anti-viral immunity. Clonal Mast Cells Activation Disorders (cMCADs) such as mastocytosis and clonal mast cells activation syndrome are characterized by an abnormal mast cells accumulation and/or activation. No data have been published on the anti-viral immune response of patients with cMCADs. The aims of the study were to collected, in a comprehensive way, outcomes of cMCADs patients who experienced a biologically-proven COVID-19 and to characterize both anti-endemic coronaviruses and specific anti-SARS-CoV-2 immune responses in these patients. Clinical follow-up and outcome data were collected prospectively for one year within the French rare disease network CEREMAST encompassing patients from all over the country. Anti-SARS-CoV-2 and anti-endemic coronaviruses specific T-cells were assessed with an enzyme-linked immunospot assay (EliSpot) and anti-SARS-CoV-2 humoral response with dosage of circulating levels of specific IgG, IgA and neutralizing antibodies. Overall, 32 cMCADs patients were identified. None of them required non-invasive or mechanical ventilation; two patients were hospitalized to receive oxygen and steroid therapy. In 21 patients, a characterization of the SARS-CoV-2-specific immune response has been performed. A majority of patients showed a high proportion of circulating SARS-CoV-2-specific interferon (IFN)-γ producing T-cells and high levels of anti-Spike IgG antibodies with neutralizing activity. In addition, no defects in anti-endemic coronaviruses responses were found in patients with cMCADs compared to non-cMCADs controls. Patients with cMCADs frequently showed a spontaneous IFN-γ T-cell production in absence of any stimulation that correlated with circulating basal tryptase levels, a marker of mast cells burden. These findings underscore that patients with cMCADs might be not at risk of severe COVID-19 and the spontaneous IFN-γ production might explain this observation.Author SummaryMast cells are immune cells involved in many biological processes including the anti-microbial response. However, previous studies suggest that mast cells may have a detrimental role in the response against viruses such as SARS-CoV-2, responsible for COVID-19. When a mutation occurs in mast cells, it can lead to a group of diseases called clonal mast cells activation disorders (cMCADs), characterized by deregulated activation of these cells. Hence, patients with cMCADs might be more susceptible to severe COVID-19 than general population.We therefore conducted a 1-year study in France to collect data from all cMCADs patients included in the CEREMAST rare disease French network and who experienced COVID-19. Interestingly, we did not find any severe COVID-19 (i.e. requiring non-invasive or mechanical ventilation) in spite of well-known risk factors for severe COVID-19 in a part of cMCADs patients.We then have studied the immune response against SARS-CoV-2 and other endemic coronaviruses in these patients. We did not observe any abnormalities in the immune response either at the level of T and B lymphocytes. These findings underscore that these patients might not be at risk of severe COVID-19 as one might have feared.

Published
2021
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9. COVID-19 outcomes in patients with inflammatory rheumatic and musculoskeletal diseases treated with rituximab: a cohort study

Author

Jérôme Avouac, Elodie Drumez, Eric Hachulla, Raphaèle Seror, Sophie Georgin-Lavialle, Soumaya El Mahou, Edouard Pertuiset, Thao Pham, Hubert Marotte, Amélie Servettaz, Fanny Domont, Pascal Chazerain, Mathilde Devaux, Pascal Claudepierre, Vincent Langlois, Arsène Mekinian, Alexandre Thibault Jacques Maria, Béatrice Banneville, Bruno Fautrel, Jacques Pouchot, Thierry Thomas, René-Marc Flipo, Christophe Richez, Florence Aeschlimann, Christian Agard, Nassim Ait-Abdallah, Jean-David Albert, Didier Alcais, Jean-Sébastien Allain, Yannick Allanore, Blanca Amador-Borreiro, Zahir Amoura, Emma Andre, Anaïs Arbault, Jean-Benoît Arlet, Laurent Arnaud, Denis Arniaud, Herliette Arty-Hue, Lucie Atlan, Alexandra Audemard-Verger, Christine Audoin-Pajot, Victor Audren, Maxime Bach-Bunner, Hélène Bacquet-Deschryver, Brigitte Bader-Meunier, Nathalie Balandraud, Jean-Charles Balblanc, Stéphane Bally, Frédéric Banal, Pierre Barbery, Thomas Barnetche, Audre Barrelet, André Basch, Vincent Baumier, Guillaume Bayer, Sophie Bayle, Catherine Beauvais, Rudie Beinat, Véronique Belin, Rakiba Belkhir, Ruben Benainous, Alexandre Belot, Mohammed Benammar, Mathilde Benhamou, Ygal Benhamou, Ahmed Benmansour, Pascal Bennet, Brigitte Bernoux-Manat, Elise Berthet, Emilie Berthoux, Ewa Bertolini, Adrien Bigot, Aurélia Bisson-Vaivre, Gilles Blaison, Gilles Bolla, Olivier Bonidan, Christine Bonnet, Raphaël Borie, Marie Bossert, Laurence Boudou, Françoise Bouhour, Kévin Bouiller, Bastien Bouldoires, Karima Boussoualim, Eric Bouvard, Regine Brondino, Pierre Buchlin, Laurence Cabantous, Patrice Cacoub, Simon Cadiou, Maurizio Carteni, Aurélia Carbasse, Brice Castel, Pascal Cathebras, Hervé Caumont, Annalisa Celant, Benjamin Chaigne, Benoît Chaillous, Romuald Champy, Agnès Charcot, Pierre Charles, Isabelle Charlot-Lambrecht, Caroline Charpin, Emmanuel Chatelus, Bernard Chaudier, Pascale Chertok, Xavier Chevalier, Maxime Chevreau, Emilie Chotard, Delphine Chu Miow Lin, Gaëlle Clavel, Cyril Clavel-Osorio, Fleur Cohen, Gregory Cohen, Marie-Eve Colette-Cedoz, Nived Collercandy, Antoine Colombey, Chloé Comarmond, Bernard Combe, Céline Comparon, Elodie Constant, Pascal Coquerelle, Justine Corli, Clémence Corre, Nathalie Costedoat-Chalumeau, Marie Couret, Natacha Courvoisier, Fabienne Coury-Lucas, Cécile Coutarel, Fabrice Coutier, Richard Damade, Laurence Daver-Malaterre, Sarahe Dehimat, Michel Delahousse, Emilie Barrois-Delattre, Delphine Denarie, Camille Deprouw, Emanuelle Dernis, Alban Deroux, Renaud Desbarbieux, Elise Descamps, Chantal Deslandre, Marie Desmurs, Jacques Despaux, Marie Desplats, Frédérick Detree, Valérie Devauchelle-Pensec, Robin Dhote, Philippe Dieude, Yannick Dieudonne, Elisabeth Diot, Guillaume Direz, Djamal-Dine Djeddi, Sarah Douvier, Béatrice Drouet, Catherine Duc, Angélique Ducornet, Carine Dufauret-Lombard, Alain Duhamel, Cécile Dumaine, Anne-Elisabeth Dumel, Chantal Dumoulin-Richez, Agnès Duquesne, Géraldine Durand, Mariane Durandin-Truffinet, Pierre-Marie Duret, Maïka Duval, Mikaël Ebbo, Esther Ebstein, Andra Economu-Dubosc, Stéphanie Emilie, Romain Euvrard, Philippe Evon, Sylvie Fabre, Dorothée Fagedet, Meryem Farhat, Marion Fauconier, Jacques Fechtenbaum, Renaud Felten, Fanny Fernandes, Nicole Ferreira-Maldent, Elodie Feurer, Amandine Fichet, Françoise Flaisler, Nans Florens, Violaine Foltz, Elisabeth Fontanges, Jennifer Foret, Anne-Claire Fougerousse, Anne Fouque-Aubert, Catherine Foutrier-Morello, Hélène Francois-Pradier, Léa Frantzen, Pierre Fritz, Antoine Froissart, Jean Fulpin, Piera Fuzibet, Francis Gaches, Laurence Gagneux-Lemoussu, Mélanie Penhoat-Gahier, Joris Galland, Frédérique Gandjbakhch, Nicole Garnier, Thomas Garraud, Jean-François Garrot, Romain Gastaldi, Véronique Gaud-Listrat, Maud Gauthier-Prieur, Dana Georgescu, Nathalie Gerard, Elisabeth Gervais, Christelle Gibert, Eric Gibert, Ghislaine Gill, Jérôme Gillard, Mélanie Gilson, Pauline Gimonnet, Jeanine-Sophie Giraudet-Le Quintrec, Aude Giraud-Morelet, Baptiste Glace, Camille Glanowski, Bertrand Godeau, Bruno Gombert, Camille Gonnet-Gracia, Tiphaine Goulenok, Philippe Goupille, Olivier Gourmelen, Sophie Govindaraju-Audouard, Franck Grados, Martine Grall-Lerosey, Bruno Grardel, Anne Grasland, Gilles Grateau, Monica Groza, Constance Guillaud, Séverine Guillaume, Caroline Guillibert, Xavier Guillot, Philippe Guilpain, Aline Gury, Marie-Hélène Guyot, Cécile Hacquard-Bouder, Marie-Noelle Havard, Jean-Pierre Hellier, Pascal Hennequin, Basile Henriot, Julien Henry, Véronique Hentgen, Marion Hermet, Muriel Herasse, Julie Hernandez, Miguel Hie, Pascal Hilliquin, Olivier Hinschberger, Ambre Hittinger-Roux, Jan Holubar, Christophe Hudry, Serge Huguenel, Clara Jaccard, Jean-Michel, Jacquemier, Bénédicte Jamard, Catherine Jan, Sylvie Jean, Mathieu Jouvray, Pierre-Antoine Juge, Laurent Juillard, Denis Jullien, Anna Kabala, Abdelkrim Kabchou, Ludovic Karkowski, Françoise Karman, Farid Kemiche, Jérémy Keraen, Pierre Kieffer, Isabelle Kone-Paut, Abdeldajallil Koreichi, Marie Kostine, Sylvain La Batide Alanore, Pierre Lafforgue, Sophie Lahalle, Marc Lambert, Isabelle Lambrecht, Sylvain Lanot, Aurélia Lanteri, Jean-Paul Larbre, Augustin Latourte, Christian Lavigne, Sophie Le Guen Guegan, Guillaume, Le Guenno, Diane Leguy, Agnès Lebrun, Emmanuel Ledoult, Nathalie Legoupil, Erick Legrand, Charlotte Lejeune, Olivier Leloire, Christophe Leroux, Rémi Leroy, Marie Leroy-Gouix, Tifenn Leturcq, Amélie Leurs, Céline Leveque-Michaud, François-Xavier Limbach, Frédéric Liote, Anne Lohse, Pierre Lozac'h, Virginie Lucas, Aurélie Madelon, Nadine Magy-Bertrand, Matthieu Mahevas, Hélène Maillard, Thibault Maillet, Sandrine Malochet-Guinamand, Quentin Mangon, Sylvie Marchou-Lopez, Nathalie Margarit, Thierry Marhadour, Xavier Mariette, Claire Martin, Alexis Mathian, François Maurier, Frédéric Maury, Betty Mazet-Guillaume, Arnaud Mazouyez, Hassan Mazyad, Nadia Mehsen-Cetre, Ulrich Meinzer, Isabelle Melki, Laurent Messer, Corinne Miceli, Martin Michaud, Catherine Michel, Matthias Michel, Mathilde Michon, Anne-Marie Milesi-Lecat, Anna Molto, Marie Moly, Olivier Moranne, Gautier Morel, Hugo Morel, Jacques Morel, Franck Morin, Laurence Moulinier, Guillaume Moulis, Bertrand Moura, Minh Nguyen, Sabine Nicolas-Vullierme, Hubert Nielly, Gaétane Nocturne, Aurore Nottez, Henri-Olivier Ollagnon, Isabelle Pacaud-Vitoux, Anne Pagnier, Caroline Paris, Antoine Parrot, Tristan Pascart, Yasmina Pascaud-Mansour, Lætitia Paulin, Stephan Pavy, Laurent Perard, Yves-Marie Pers, Micheline Pha, Maud Pichon, Audrey Pierreisnard, Gabrielle Pizana, Sylvaine Poignant, Elsa Poix, Agnès Portier, Antoine Poulet, Samira Plassard, Grégory Pugnet, Déborah Puyraimond-Zemmour, Pierre Quartier-Dit-Maire, Marion Quenet, Viviane Queyrel, Loïc Raffray, Philippe Remy, Myriam Renard, Jessica Rene, Sabine Revuz, Bénédicte Rey, Gaëlle Richard-Colmant, Etienne Riviere, Sébastien Riviere, Sophie Robin, Julien Rohmer, Isabelle Roitg, Mélanie Romier, Michel Rolland, Mélanie Roriz, Carole Rosenberg, Linda Rossi, Olivier Roth, Sid-Ahmed Rouidi, Mathilde Roumier, Mickaël Rousiere, Clémentine Rousselin, Bénédicte Rouviere, Christian Roux, Fabienne Roux, Marielle Roux, Nicolas Roux, Diane Rouzaud, Sylvie Rozenberg, Isabelle Sacco, Fatiha Sadji, Laurent Sailler, Carine Salliot, Jean-Hugues Salmon, Alain Saraux, Jean Schmidt, Julie Seguier, Jérémie Sellam, Eric Senbel, Thomas Sene, Patricia Senet, Pascal Seve, Aurélie Sicaud, Perrine Smets, Vincent Sobanski, Christelle Sordet, Elisabeth Sornay-Rendu, Odile Souchaud-Debouverie, Lætitia Sparsa, Lionel Spielmann, Sarah Steib, Chloé Stavris, Catherine Straus, Victor Strotz, Paulina Szafors, Séverine Taffignon-Clave, Justine Simoens, Claire Theillac, Nora Tenenbaum, Benoît Thomachot, Nathalie Tieulie, Soizic Tiriau, Alice Tison, Eric Toussirot, Ludovic Trefond, Sophie Trijau, Sébastien Trouillier, Anne-Priscille Trouvin, Marie-Elise Truchetet, Marc Ulrich, Jacques Vaquier, Eric Veillard, Laurent Veillon, Guillaume Vial, Jean-François Viallard, Judith Victor, Claire Vidon, Mathias Vidon, Camille Vigne, Alexandre Virone, Ursula Warzocha, Daniel Wendling, Claude Werle, Cécile Wibaux, Alexandra Willems, Michel Wisniewski, Juliette Woessner, Bernadette Xerri-Campano, Service de rhumatologie [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Immunologie des Maladies Virales et Autoimmunes (IMVA - U1184), Université Paris-Sud - Paris 11 (UP11)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Tourcoing, Centre Hospitalier René Dubos [Pontoise], Assistance Publique - Hôpitaux de Marseille (APHM), Santé Ingénierie Biologie Saint-Etienne (SAINBIOSE), Centre Ingénierie et Santé (CIS-ENSMSE), École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Biologie Intégrative du Tissu Osseux (LBTO), Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d'Investigation Clinique - Epidémiologie Clinique Saint-Etienne (CIC-EC), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Reims (CHU Reims), CHU Pitié-Salpêtrière [AP-HP], Groupe Hospitalier Diaconesses Croix Saint-Simon, Centre hospitalier intercommunal de Poissy/Saint-Germain-en-Laye - CHIPS [Poissy], Epidemiology in Dermatology and Evaluation in Therapeutics (EpiDermE), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), CHU Saint-Antoine [AP-HP], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), CHU Bordeaux [Bordeaux], Composantes innées de la réponse immunitaire et différenciation (CIRID), Université Bordeaux Segalen - Bordeaux 2-Centre National de la Recherche Scientifique (CNRS), Laboratoire Chrono-environnement (UMR 6249) (LCE), Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Maladies génétiques d'expression pédiatrique (U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de Médecine Interne = Hôpital de jour de médecine [CHU Tenon], INSERM U1059, SAINBIOSE - Santé, Ingénierie, Biologie, Saint-Etienne (SAINBIOSE-ENSMSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Biologie intégrative du tissu osseux, Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), CIC Saint Etienne, Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Nord (Saint Etienne), centre hospitalier intercommunal de Poissy/Saint-Germain-en-Laye - CHIPS [Poissy], Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Laboratoire Chrono-environnement - CNRS - UBFC (UMR 6249) (LCE), Service de rhumatologie, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Service de Médecine Interne [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de rhumatologie [CH Gustave Dron, Tourcoing], Centre Hospitalier Gustave Dron [Tourcoing], Hopital Réné Dubos, Université Paris 1 Panthéon-Sorbonne - UFR Science Politique (UP1 UFR11), and Université Paris 1 Panthéon-Sorbonne (UP1)

Subjects
medicine.medical_specialty, Immunology, law.invention, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, immune system diseases, law, hemic and lymphatic diseases, Internal medicine, Clinical endpoint, Immunology and Allergy, Medicine, 030212 general & internal medicine, 030203 arthritis & rheumatology, business.industry, Hazard ratio, Odds ratio, Articles, medicine.disease, Intensive care unit, 3. Good health, [SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology, Rheumatoid arthritis, Cohort, Rituximab, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Cohort study, medicine.drug
Abstract

Summary Background Various observations have suggested that the course of COVID-19 might be less favourable in patients with inflammatory rheumatic and musculoskeletal diseases receiving rituximab compared with those not receiving rituximab. We aimed to investigate whether treatment with rituximab is associated with severe COVID-19 outcomes in patients with inflammatory rheumatic and musculoskeletal diseases. Methods In this cohort study, we analysed data from the French RMD COVID-19 cohort, which included patients aged 18 years or older with inflammatory rheumatic and musculoskeletal diseases and highly suspected or confirmed COVID-19. The primary endpoint was the severity of COVID-19 in patients treated with rituximab (rituximab group) compared with patients who did not receive rituximab (no rituximab group). Severe disease was defined as that requiring admission to an intensive care unit or leading to death. Secondary objectives were to analyse deaths and duration of hospital stay. The inverse probability of treatment weighting propensity score method was used to adjust for potential confounding factors (age, sex, arterial hypertension, diabetes, smoking status, body-mass index, interstitial lung disease, cardiovascular diseases, cancer, corticosteroid use, chronic renal failure, and the underlying disease [rheumatoid arthritis vs others]). Odds ratios and hazard ratios and their 95% CIs were calculated as effect size, by dividing the two population mean differences by their SD. This study is registered with ClinicalTrials.gov, NCT04353609. Findings Between April 15, 2020, and Nov 20, 2020, data were collected for 1090 patients (mean age 55·2 years [SD 16·4]); 734 (67%) were female and 356 (33%) were male. Of the 1090 patients, 137 (13%) developed severe COVID-19 and 89 (8%) died. After adjusting for potential confounding factors, severe disease was observed more frequently (effect size 3·26, 95% CI 1·66–6·40, p=0·0006) and the duration of hospital stay was markedly longer (0·62, 0·46–0·85, p=0·0024) in the 63 patients in the rituximab group than in the 1027 patients in the no rituximab group. 13 (21%) of 63 patients in the rituximab group died compared with 76 (7%) of 1027 patients in the no rituximab group, but the adjusted risk of death was not significantly increased in the rituximab group (effect size 1·32, 95% CI 0·55–3·19, p=0·53). Interpretation Rituximab therapy is associated with more severe COVID-19. Rituximab will have to be prescribed with particular caution in patients with inflammatory rheumatic and musculoskeletal diseases. Funding None.

Published
2021
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10. Efficacy and safety of Infliximab in systemic sarcoidosis according to GenPhenReSa organ-involvement phenotype: a retrospective study of 55 patients

Author

Etienne Rivière, Wendy Jourde, Noémie Gensous, Xavier Demant, Emmanuel Ribeiro, Pierre Duffau, Patrick Mercié, Jean-François Viallard, and Estibaliz Lazaro

Subjects
Sarcoidosis, Infliximab, Disease clusters, Infection, Diseases of the respiratory system, RC705-779
Abstract

Abstract Background Infliximab is currently recommended as a third-line treatment for refractory sarcoidosis. Data in function of clinical phenotype are currently lacking. We evaluated patients’ characteristics and responses to infliximab according to their GenPhenReSa cluster. Methods We evaluated clinical and biological characteristics of patients diagnosed with sarcoidosis who received infliximab between September 2008 and April 2019 at our centre. Results Fifty-five patients (median disease duration, 87 months) received infliximab: 48 (87%) as a second- or third-line treatment, and 7 (13%) as a first-line treatment. After a median duration of 12 months, 24 (45%) and 14 (25%) patients achieved complete and partial responses, respectively, together with a significant decrease in the number of affected organs and tapering of steroid doses. All patients with neurosarcoidosis (OR 17), 90% in group 2 (ocular-cardiac-cutaneous-CNS, OR 7.4), and approximately two-thirds of those in groups 1 (abdominal organs), 4 (pulmonary-lympho-nodal), and 5 (extrapulmonary), achieved a response, whereas patients in group 3 (musculoskeletal-cutaneous) had a treatment-failure OR of 9. Infliximab could be stopped after complete remission was achieved in 7 patients: 4 relapsed after a median of 6 months. Overall, 36% of patients experienced serious adverse events, mainly infections, which led to treatment cessation in 29% of patients and caused two deaths. Conclusions Other than patients with musculoskeletal-cutaneous involvement (group 3), infliximab led to a good response for patients with CNS (group 2) and liver (group 1) organ-predominant sarcoidosis. However, it led to serious infections and merely suspended sarcoidosis, so further research on factors predictive of relapse is needed.

Published
2024
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11. Effective Anti–SARS-CoV-2 Immune Response in Patients With Clonal Mast Cell Disorders

Author

Julien Rossignol, Amani Ouedrani, Cristina Bulai Livideanu, Stéphane Barete, Louis Terriou, David Launay, Richard Lemal, Celine Greco, Laurent Frenzel, Cecile Meni, Christine Bodemere-Skandalis, Laura Polivka, Anne-Florence Collange, Hassiba Hachichi, Sonia Bouzourine, Djazira Nait Messaoud, Mathilde Negretto, Laurence Vendrame, Marguerite Jambou, Marie Gousseff, Stéphane Durupt, Jean-Christophe Lega, Jean-Marc Durand, Caroline Gaudy, Gandhi Damaj, Marie-Pierre Gourin, Mohamed Hamidou, Laurence Bouillet, Edwige Le Mouel, Alexandre Maria, Patricia Zunic, Quentin Cabrera, Denis Vincent, Christian Lavigne, Etienne Riviere, Clement Gourguechon, Marie Courbebaisse, David Lebeaux, Béatrice Parfait, Gérard Friedlander, Anne Brignier, Ludovic Lhermitte, Thierry Jo Molina, Julie Bruneau, Julie Agopian, Patrice Dubreuil, Dana Ranta, Alexandre Mania, Michel Arock, Isabelle Staropoli, Olivier Tournilhac, Olivier Lortholary, Olivier Schwartz, Lucienne Chatenoud, Olivier Hermine, Centre de référence des mastocytoses (CEREMAST), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Référence des Mastocytoses de Toulouse (CEREMAST), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre de Recherche en Cancérologie de Marseille (CRCM), Aix Marseille Université (AMU)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Role of intra-Clonal Heterogeneity and Leukemic environment in ThErapy Resistance of chronic leukemias (CHELTER), Université Clermont Auvergne (UCA), Virus et Immunité - Virus and immunity (CNRS-UMR3569), Institut Pasteur [Paris] (IP)-Centre National de la Recherche Scientifique (CNRS), The authors would like to thank Fondation Université de Paris, AXA research fund, Fondation Hôpitaux de Paris-Hôpitaux de France, Mécénat du GH APHP. CUP, Fondation pour la Recherche en Physiologie and DMU BioPhyGen for the funding of the COVID-HOP study., ANR-20-COV1-0001,APCOD,Les cellules présentatrices d'antigènes dans la maladie de COVID-19 à résolution monocellulaire(2020), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPC), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPC), CHU Toulouse [Toulouse], Virus et Immunité - Virus and immunity, and Institut Pasteur [Paris]-Centre National de la Recherche Scientifique (CNRS)

Subjects
PBMC, peripheral blood mononuclear cell, BMI, body mass index, T cells, B-cells, CEREMAST, Centre de Référence des Mastocytoses, CM, cutaneous mastocytosis, MMAS, monoclonal mast cell activation syndrome, PHA, phytohemagglutinin, PMSI, Programme de médicalisation des systèmes d'information, Antibodies, Viral, SARS-CoV-2, severe acute respiratory syndrome coronavirus 2, Antiviral Agents, WHO, World Health Organization, MIS, mastocytosis in the skin, HEK, human embryonic kidney, Humans, Immunology and Allergy, IFN, interferon, Mast Cells, APHP, Assistance Publique - Hôpitaux de Paris (Paris Public Hospital Group), B cells, SARS-CoV-2, T-cells, Immunity, COVID-19, ISM, indolent systemic mastocytosis, Clonal mast cell activation syndrome, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, MCAS, mast cell activation syndrome, Original Article, Mast cell activation dis- orders, SSM, smoldering systemic mastocytosis, NK, natural killer, Mastocytosis, COVID-19, coronavirus disease 19, Mast cell activation disorders, cMCAD, clonal mast cell activation disorder
Abstract

International audience; BackgroundMast cells are key players in innate immunity and the TH2 adaptive immune response. The latter counterbalances the TH1 response, which is critical for antiviral immunity. Clonal mast cell activation disorders (cMCADs, such as mastocytosis and clonal mast cell activation syndrome) are characterized by abnormal mast cell accumulation and/or activation. No data on the antiviral immune response in patients with MCADs have been published.ObjectiveTo study a comprehensive range of outcomes in patients with cMCAD with PCR- or serologically confirmed coronavirus disease 2019 and to characterize the specific anti–severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) immune response in this setting.MethodsClinical follow-up and outcome data were collected prospectively over a 12-month period by members of the French Centre de Référence des Mastocytoses rare disease network. Anti–SARS-CoV-2–specific T-cell activity was measured with an ELISA, and humoral responses were evaluated by assaying circulating levels of specific IgG, IgA, and neutralizing antibodies.ResultsOverall, 32 patients with cMCAD were evaluated. None required noninvasive or mechanical ventilation. Two patients were admitted to hospital for oxygen and steroid therapy. The SARS-CoV-2–specific immune response was characterized in 21 of the 32 patients. Most had high counts of circulating SARS-CoV-2–specific, IFN-γ–producing T cells and high titers of neutralizing antispike IgGs. The patients frequently showed spontaneous T-cell IFN-γ production in the absence of stimulation; this production was correlated with basal circulating tryptase levels (a marker of the mast cell burden).ConclusionsPatients with cMCADs might not be at risk of severe coronavirus disease 2019, perhaps due to their spontaneous production of IFN-γ.

Published
2022
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12. Clinical phenotype of adult-onset systemic histiocytosis harboring BRAF in-frame deletions

Author

Matthias Papo, Jérôme Razanamahéry, Malik Da Silva, Zofia Hélias-Rodzewicz, Vsevolod Potapenko, Suzanna Bota, Vanessa Leguy-Seguin, Stéphane Dominique, Raphaël Lhote, Quentin Moyon, Dov Taïeb, Tom Abrassart, Marion Campana, Visal Keo, Etienne Rivière, Olivier Lucidarme, Fleur Cohen-Aubart, Zahir Amoura, Julien Haroche, and Jean-François Emile

Subjects
Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Not available.

Published
2024
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13. Distributed Applications and Interoperable Systems : 18th IFIP WG 6.1 International Conference, DAIS 2018, Held As Part of the 13th International Federated Conference on Distributed Computing Techniques, DisCoTec 2018, Madrid, Spain, June 18-21, 2018, Proceedings

Author

Silvia Bonomi, Etienne Rivière, Silvia Bonomi, and Etienne Rivière

Subjects
Application software, Data protection, Operating systems (Computers), Software engineering, Computer networks, Computer systems
Abstract

This book constitutes the proceedings of the 18th IFIP International Conference on Distributed Applications and Interoperable Systems, DAIS 2018, held in Madrid, Spain, in June 2018. The 10 papers presented together with 2 short papers in this volume were carefully reviewed and selected from 33 submissions. The papers are organized in topical sections on application domains, including stream processing, video dissemination, storage, privacy protection, and large-scale orchestration.

Published
2018

14. Utility of indium-111 platelet scintigraphy for understanding the mechanism of thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia

Author

Pauline Durand, Valérie Pottier, Charles Mesguich, Frédéric Debordeaux, Estibaliz Lazaro, Jean-François Viallard, and Etienne Rivière

Subjects
Myelodysplastic syndrome, Immune thrombocytopenia, Indium-111 platelet scintigraphy, Diseases of the blood and blood-forming organs, RC633-647.5, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Abstract Background Thrombocytopenia occurs in 60% of patients with myelodysplastic syndromes (MDS), increasing the risk of life-threatening haemorrhage in this population of mainly old patients with comorbidities. However, data are scare regarding immune thrombocytopenia (ITP) secondary to MDS. Aim We analyzed the utility of indium-111 platelet scintigraphy (IPS) to better characterize the mechanisms of thrombocytopenia in 21 adult patients with MDS. Methods Adult patients with a definite diagnosis of MDS according to the international criteria who underwent IPS between 2009 and 2018 because of an increased bleeding risk were retrospectively selected. Autologous 111Indium platelet labelling was performed with a technique similar to that described previously using a standardized method. Results Platelet lifespan ≤ 6 days identified patients with peripheral platelet destruction. Taking into account the response to ITP-directed therapies after IPS, the sensitivity, specificity, and positive and negative predictive values of IPS were 100%, 84.6%, 80%, and 100%, respectively. Conclusion We show that IPS can be a useful tool to identify the mechanism and guide treatment of a chronic thrombocytopenia increasing the bleeding risk in patients with MDS.

Published
2023
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15. Machine‐as‐a‐Service: Blockchain‐based management and maintenance of industrial appliances

Author

Viet Hoang Tran, Bernard Lenssens, Ayham Kassab, Alexis Laks, Etienne Rivière, Guillaume Rosinosky, and Ramin Sadre

Subjects
blockchain, industry, IoT, Machine‐as‐a‐Service, telemetry, Engineering (General). Civil engineering (General), TA1-2040, Electronic computers. Computer science, QA75.5-76.95
Abstract

Abstract Machine‐as‐a‐Service (MaaS) is an emerging service model for industrial appliances. With MaaS, machines are rented instead of being acquired, and their lifecycle is handled by an ecosystem of specialized actors, such as different independent maintenance companies certified for interventions on specific hardware. As the number of actors, clients, and providers involved in a MaaS ecosystem grows, maintaining mutual trust relationships between all involved parties and orchestrating MaaS operations in centralized fashion quickly becomes intractable. We present a blockchain‐based approach to providing MaaS in industrial settings where rented machines are equipped with IoT sensors, and where MaaS operations are orchestrated in a transparent, decentralized, and scalable way using a collection of smart contracts deployed over an infrastructure combining the Ethereum and InterPlanetary File System decentralized services. We detail the operations of MaaS, such as the lifecycle of management operations, and report on the performance of a prototype implementation deployed in the cloud.

Published
2023
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16. Myasthenia gravis and paroxysmal nocturnal hemoglobinuria after thymectomy: A rare association

Author

Jean Galtier, Fanny Duval, Irène Machelart, Carine Greib, Estibaliz Lazaro, Jean‐Luc Pellegrin, Jean‐François Viallard, Regis Peffault de laTour, and Etienne Rivière

Subjects
aplastic anemia, autoimmune disease, immunohematology, Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Abstract Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare clonal autoimmune disease manifesting with hemolysis, thrombosis, or bone marrow failure. We present an atypical association of myasthenia gravis, aplastic anemia, and PNH occurring years after thymectomy. While this association might be extremely rare, it may not be coincidental as there is a common pathophysiology between PNH and aplastic anemia, with the latter reported in several thymoma/thymectomy cases. Eculizumab was introduced with good efficacy and without safety concern in our patient, leading to long‐term control of PNH without worsening of myasthenia gravis.

Published
2021
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17. Prevention of infection in asplenic adult patients by general practitioners in France between 2013 and 2016

Author

Charlotte Quéffélec, Louis Billet, Pierre Duffau, Estibaliz Lazaro, Irène Machelart, Carine Greib, Jean-François Viallard, Jean-Luc Pellegrin, and Etienne Rivière

Subjects
Splenectomy, General practice, Infections, Vaccination, Medicine (General), R5-920
Abstract

Abstract Background Guidelines that detail preventive measures against Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae type b, and influenza are published annually in France to decrease the risk of severe infections in immunocompromised patients. We aimed at describing adherence to these guidelines by GPs in the management of their asplenic patients in France between 2013 and 2016. Method We conducted a multicenter retrospective study between January 2013 and December 2016 in three French hospitals: asplenic adults were identified and their GPs were questioned. A descriptive analysis was performed to identify the immunization coverage, type and length of antibiotic prophylaxis, number of infectious episodes, and education of patients. Results 103 patients were finally included in this study: only 57% were adequately vaccinated against Streptococcus pneumoniae or Neisseria meningitidis, 74% against Haemophilus influenzae type b, and 59% against influenza. Only 24% of patients received a combination of all four vaccinations. Two-thirds of patients received prophylactic antibiotics for at least 2 years. Overall, this study found that 50% of splenectomized patients experienced at least one pulmonary or otorhinolaryngological infection, or contracted influenza. Conclusions These data match those reported in other countries, including Australia and the United Kingdom, meaning a still insufficient coverage of preventive measures in asplenic patients. Improved medical data sharing strategies between healthcare professionals, along with educational measures to keep patients and physicians up to date in the prevention of infections after splenectomy would improve health outcomes of asplenic patients.

Published
2020
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18. Plasma Markers of Neutrophil Extracellular Trap Are Linked to Survival but Not to Pulmonary Embolism in COVID-19-Related ARDS Patients

Author

Renaud Prével, Annabelle Dupont, Sylvie Labrouche-Colomer, Geoffrey Garcia, Antoine Dewitte, Antoine Rauch, Julien Goutay, Morgan Caplan, Elsa Jozefowicz, Jean-Philippe Lanoix, Julien Poissy, Etienne Rivière, Arthur Orieux, Denis Malvy, Didier Gruson, Loic Garçon, Sophie Susen, and Chloé James

Subjects
neutrophil extracellular trap, acute respiratory distress syndrome, COVID-19, immunothrombosis, pulmonary embolism, Immunologic diseases. Allergy, RC581-607
Abstract

IntroductionCoronavirus disease 2019 (COVID-19) can cause life-threatening acute respiratory distress syndrome (ARDS). Recent data suggest a role for neutrophil extracellular traps (NETs) in COVID-19-related lung damage partly due to microthrombus formation. Besides, pulmonary embolism (PE) is frequent in severe COVID-19 patients, suggesting that immunothrombosis could also be responsible for increased PE occurrence in these patients. Here, we evaluate whether plasma levels of NET markers measured shorty after admission of hospitalized COVID-19 patients are associated with clinical outcomes in terms of clinical worsening, survival, and PE occurrence.Patients and MethodsNinety-six hospitalized COVID-19 patients were included, 50 with ARDS (severe disease) and 46 with moderate disease. We collected plasma early after admission and measured 3 NET markers: total DNA, myeloperoxidase (MPO)–DNA complexes, and citrullinated histone H3. Comparisons between survivors and non-survivors and patients developing PE and those not developing PE were assessed by Mann–Whitney test.ResultsAnalysis in the whole population of hospitalized COVID-19 patients revealed increased circulating biomarkers of NETs in patients who will die from COVID-19 and in patients who will subsequently develop PE. Restriction of our analysis in the most severe patients, i.e., the ones who enter the hospital for COVID-19-related ARDS, confirmed the link between NET biomarker levels and survival but not PE occurrence.ConclusionOur results strongly reinforce the hypothesis that NETosis is an attractive therapeutic target to prevent COVID-19 progression but that it does not seem to be linked to PE occurrence in patients hospitalized with COVID-19.

Published
2022
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19. A new tool for assessing short debriefings after immersive simulation: validity of the SHORT scale

Author

Etienne Rivière, Etienne Aubin, Samuel-Lessard Tremblay, Gilles Lortie, and Gilles Chiniara

Subjects
Simulation training, High-fidelity simulation training, Patient simulation, Educational measurement, Formative feedback, Generalizability theory, Special aspects of education, LC8-6691, Medicine
Abstract

Abstract Background Simulation is being increasingly used worldwide in healthcare education. However, it is costly both in terms of finances and human resources. As a consequence, several institutions have designed programs offering several short immersive simulation sessions, each followed by short debriefings. Although debriefing is recommended, no tool exists to assess appropriateness of short debriefings after such simulation sessions. We have developed the Simulation in Healthcare retrOaction Rating Tool (SHORT) to assess short debriefings, and provide some validity evidence for its use. Methods We designed this scale based on our experience and previously published instruments, and tested it by assessing short debriefings of simulation sessions offered to emergency medicine residents at Laval University (Canada) from 2015 to 2016. Analysis of its reliability and validity was done using Standards for educational and psychological testing. Generalizability theory was used for testing internal structure evidence for validity. Results Two raters independently assessed 22 filmed short debriefings. Mean debriefing length was 10:35 (min 7:21; max 14:32). Calculated generalizability (reliability) coefficients are φ = 0.80 and φ-λ3 = 0.82. The generalizability coefficient for a single rater assessing three debriefings is φ = 0.84. Conclusions The G study shows a high generalizability coefficient (φ ≥ 0.80), which demonstrates a high reliability. The response process evidence for validity provides evidence that no errors were associated with using the instrument. Further studies should be done to demonstrate validity of the English version of the instrument and to validate its use by novice raters trained in the use of the SHORT.

Published
2019
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20. Covichem: A biochemical severity risk score of COVID-19 upon hospital admission.

Author

Marie-Lise Bats, Benoit Rucheton, Tara Fleur, Arthur Orieux, Clément Chemin, Sébastien Rubin, Brigitte Colombies, Arnaud Desclaux, Claire Rivoisy, Etienne Mériglier, Etienne Rivière, Alexandre Boyer, Didier Gruson, Isabelle Pellegrin, Pascale Trimoulet, Isabelle Garrigue, Rana Alkouri, Charles Dupin, François Moreau-Gaudry, Aurélie Bedel, and Sandrine Dabernat

Subjects
Medicine, Science
Abstract

Clinical and laboratory predictors of COVID-19 severity are now well described and combined to propose mortality or severity scores. However, they all necessitate saturable equipment such as scanners, or procedures difficult to implement such as blood gas measures. To provide an easy and fast COVID-19 severity risk score upon hospital admission, and keeping in mind the above limits, we sought for a scoring system needing limited invasive data such as a simple blood test and co-morbidity assessment by anamnesis. A retrospective study of 303 patients (203 from Bordeaux University hospital and an external independent cohort of 100 patients from Paris Pitié-Salpêtrière hospital) collected clinical and biochemical parameters at admission. Using stepwise model selection by Akaike Information Criterion (AIC), we built the severity score Covichem. Among 26 tested variables, 7: obesity, cardiovascular conditions, plasma sodium, albumin, ferritin, LDH and CK were the independent predictors of severity used in Covichem (accuracy 0.87, AUROC 0.91). Accuracy was 0.92 in the external validation cohort (89% sensitivity and 95% specificity). Covichem score could be useful as a rapid, costless and easy to implement severity assessment tool during acute COVID-19 pandemic waves.

Published
2021
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22. Antigenic Mimicry in Paraneoplastic Immune Thrombocytopenia

Author

Guillaume Vial, Etienne Rivière, Anne-Aurélie Raymond, Chloé James, Sylvaine Di-Tommaso, Nathalie Dugot-Senant, Jean-William Dupuy, Mokrane Yacoub, Marie Parrens, Fréderic Saltel, and Jean-François Viallard

Subjects
antigenic mimicry, solid tumors, tumor antigens, immune thrombocytopenia, proteomics, Immunologic diseases. Allergy, RC581-607
Abstract

The association of immune thrombocytopenia (ITP) with cancer has been reported, but the causality of tumor cells in paraneoplastic ITP pathogenesis and maintenance has never been established. We analyzed the unusual case of refractory ITP and coincident urothelial tumor of the kidney with circulating high titer anti-GPIIBIIIA autoantibodies. Intriguingly, after nephrectomy, the patient recovered fully and her anti-GPIIBIIIA autoantibodies disappeared. Proteomic and immunohistochemistry analyses revealed erratic GPIIB expression by the tumor cells, suggesting possible antigenic mimicry chronically stimulating the immune system and leading to this patient's refractory ITP. Such previously unreported findings provide proof-of-concept that requires further confirmation with the prospective study of a larger number of patients.

Published
2019
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