Alexandre Bélot

ORCID: 0000-0003-4902-5332
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Research Areas
  • Autoimmune and Inflammatory Disorders Research
  • Inflammasome and immune disorders
  • Systemic Lupus Erythematosus Research
  • Immunodeficiency and Autoimmune Disorders
  • Kawasaki Disease and Coronary Complications
  • interferon and immune responses
  • Adolescent and Pediatric Healthcare
  • COVID-19 Clinical Research Studies
  • Immune Cell Function and Interaction
  • SARS-CoV-2 and COVID-19 Research
  • Otitis Media and Relapsing Polychondritis
  • Diabetes and associated disorders
  • Vasculitis and related conditions
  • Renal Diseases and Glomerulopathies
  • T-cell and B-cell Immunology
  • Inflammatory Myopathies and Dermatomyositis
  • Immune Response and Inflammation
  • Long-Term Effects of COVID-19
  • Respiratory viral infections research
  • Coagulation, Bradykinin, Polyphosphates, and Angioedema
  • Mechanical Circulatory Support Devices
  • Atherosclerosis and Cardiovascular Diseases
  • Acute Lymphoblastic Leukemia research
  • Lymphoma Diagnosis and Treatment
  • Parvovirus B19 Infection Studies

Hospices Civils de Lyon
2016-2025

Centre International de Recherche en Infectiologie
2016-2025

Hôpital Femme Mère Enfant
2016-2025

Université Claude Bernard Lyon 1
2016-2025

Centre National de la Recherche Scientifique
2016-2025

Inserm
2016-2025

École Normale Supérieure de Lyon
2016-2025

The Lymphoma Academic Research Organisation
2021-2025

ERN GUARD-Heart
2025

Hôpital Lyon Sud
2018-2024

Paul Bastard Lindsey B. Rosen Qian Zhang Eleftherios Michailidis Hans-Heinrich Hoffmann and 95 more Yu Zhang Karim Dorgham Quentin Philippot Jérémie Rosain Vivien Béziat Jérémy Manry Elana Shaw Liis Haljasmägi Pärt Peterson Lazaro Lorenzo Lucy Bizien Sophie Trouillet‐Assant Kerry Dobbs Adriana A. de Jesus Alexandre Bélot Anne Kallaste Émilie Catherinot Yacine Tandjaoui-Lambiotte Jérémie Le Pen Gaspard Kerner Benedetta Bigio Yoann Seeleuthner Rui Yang Alexandre Bolze András N. Spaan Ottavia M. Delmonte Michael S. Abers Alessandro Aiuti Giorgio Casari Vito Lampasona Lorenzo Piemonti Fabio Ciceri Kaya Bilgüvar Richard P. Lifton Marc Vasse David M. Smadja Mélanie Migaud Jérôme Hadjadj Benjamin Terrier Darragh Duffy Lluís Quintana‐Murci Diederik van de Beek Lucie Roussel Donald C. Vinh Stuart G. Tangye Filomeen Haerynck David Dalmau Javier Martínez‐Picado Petter Brodin Michel C. Nussenzweig Stéphanie Boisson‐Dupuis Carlos Rodríguez‐Gallego Guillaume Vogt Trine H. Mogensen Andrew J. Oler Jingwen Gu Peter D. Burbelo Jeffrey I. Cohen Andrea Biondi Laura Rachele Bettini Mariella D’Angiò Paolo Bonfanti Patrick Rossignol Julien Mayaux Frédéric Rieux‐Laucat Eystein S. Husebye Francesca Fusco Matilde Valeria Ursini Luisa Imberti Alessandra Sottini Simone Paghera Eugenia Quirós-Roldán Camillo Rossi Riccardo Castagnoli Daniela Montagna Amelia Licari Gian Luigi Marseglia Xavier Duval Jade Ghosn John S. Tsang Raphaela Goldbach‐Mansky Kai Kisand Michail S. Lionakis Anne Puel Shen‐Ying Zhang Steven M. Holland Guy Gorochov Emmanuelle Jouanguy Charles M. Rice Aurélie Cobat Luigi D. Notarangelo Laurent Abel Helen C. Su Jean‐Laurent Casanova Andrés A. Arias

Interindividual clinical variability in the course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is vast. We report that at least 101 987 patients with life-threatening disease 2019 (COVID-19) pneumonia had neutralizing immunoglobulin G (IgG) autoantibodies (auto-Abs) against interferon-ω (IFN-ω) (13 patients), 13 types IFN-α (36), or both (52) onset critical disease; a few also auto-Abs other three type I IFNs. The neutralize ability corresponding IFNs to block...

10.1126/science.abd4585 article EN cc-by Science 2020-09-24

<h3>Objectives</h3> To validate the previously proposed classification criteria for Henoch–Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). <h3>Methods</h3> Step 1: retrospective/prospective web-data collection children with HSP, c-PAN, c-WG c-TA age at diagnosis ≤18 years. 2: blinded by consensus panel of a representative sample 280 cases. 3: statistical (sensitivity, specificity, area under curve κ-agreement)...

10.1136/ard.2009.116657 article EN Annals of the Rheumatic Diseases 2010-04-22
Paul Bastard Adrian Gervais Tom Le Voyer Jérémie Rosain Quentin Philippot and 95 more Jérémy Manry Eleftherios Michailidis Hans-Heinrich Hoffmann Shohei Eto Marina García-Prat Lucy Bizien Alba Parra-Martínez Rui Yang Liis Haljasmägi Mélanie Migaud Karita Särekannu Julia Maslovskaja Nicolas de Prost Yacine Tandjaoui-Lambiotte Charles‐Édouard Luyt Blanca Amador-Borrero Alexandre Gaudet Julien Poissy Pascal Morel Pascale Richard Fabrice Cognasse Jesús Troya Sophie Trouillet‐Assant Alexandre Bélot Kahina Saker Pierre Garçon Jacques G. Rivière Jean‐Christophe Lagier Stéphanie Gentile Lindsey B. Rosen Elana Shaw Tomohiro Morio Junko Tanaka David Dalmau Pierre‐Louis Tharaux D. Sène Alain Stépanian Bruno Mégarbane Vasiliki Triantafyllia Arnaud Fekkar James R. Heath José Luis Franco Juan‐Manuel Anaya Jordi Solé‐Violán Luisa Imberti Andrea Biondi Paolo Bonfanti Riccardo Castagnoli Ottavia M. Delmonte Yu Zhang Andrew L. Snow Steven M. Holland Catherine M. Biggs Marcela Moncada‐Vélez Andrés A. Arias Lazaro Lorenzo Soraya Boucherit Boubacar Coulibaly Dany Anglicheau Anna M. Planas Filomeen Haerynck Sotiriјa Duvlis Robert L. Nussbaum Tayfun Özçelık Sevgi Keleş Ahmed Aziz Bousfiha Jalila El Bakkouri Carolina Ramírez‐Santana Stéphane Paul Qiang Pan‐Hammarström Lennart Hammarström Annabelle Dupont Alina Kurolap Christine N. Metz Alessandro Aiuti Giorgio Casari Vito Lampasona Fabio Ciceri Lucila Akune Barreiros Elena Domínguez‐Garrido Mateus Vidigal Mayana Zatz Diederik van de Beek Sabina Sahanic Ivan Tancevski Yuriy Stepanovskyy Oksana Boyarchuk Yoko Nukui Miyuki Tsumura Loreto Vidaur Stuart G. Tangye Sonia Burrel Darragh Duffy Lluís Quintana‐Murci Adam Klocperk

Circulating autoantibodies (auto-Abs) neutralizing high concentrations (10 ng/mL, in plasma diluted 1 to 10) of IFN-α and/or -ω are found about 10% patients with critical COVID-19 pneumonia, but not subjects asymptomatic infections. We detect auto-Abs 100-fold lower, more physiological, (100 pg/mL, 1/10 dilutions plasma) 13.6% 3,595 COVID-19, including 21% 374 > 80 years, and 6.5% 522 severe COVID-19. These antibodies also detected 18% the 1,124 deceased (aged 20 days-99 years; mean: 70...

10.1126/sciimmunol.abl4340 article EN cc-by Science Immunology 2021-08-10
Naïm Ouldali Julie Toubiana Denise Antona Étienne Javouhey Fouad Madhi and 95 more Mathie Lorrot Pierre-Louis Léger Caroline Galeotti Caroline Claude Arnaud Wiedemann Noémie Lachaume Caroline Ovaert Morgane Dumortier Jean‐Emmanuel Kahn Alexis Mandelcwajg Lucas Percheron Blandine Biot Jeanne Bordet Marie‐Laure Girardin David Dawei Yang Marion Grimaud Mehdi Oualha Slimane Allali Fanny Bajolle Constance Beyler Ulrich Meinzer Michaël Levy Ana-Maria Paulet Yaël Levy Robert Cohen Alexandre Bélot François Angoulvant Cinthia Rames A. Donzeau Sophie Léty Cristian Fedorczuk Marion Lajus Philippe Bensaïd Yacine Laoudi Charlotte Pons Camille Beaucourt Loïc de Pontual Camille Aupiais Alain Lefèvre‐Utile Muriel Richard Etienne Goisque Xavier Iriart Olivier Brissaud Marion Bailhache Pierre Segretin Julie Molimard Marie-Clothilde Orcel Grégoire Benoist Elsa Amouyal Margaux Guerder Robin Pouyau Jean-Marie De Guillebon De Resnes Ellia Mezgueldi Fleur Cour‐Andlauer Côme Horvat Pierre Poinsot Cécile Frachette Antoine Ouziel Yves Gillet Catherine Barrey Jacques Brouard Caroline Faucon Henri Giniès Vathanaksambath Ro Narcisse Elanga Vincent Gajdos Romain Basmaci Névéna Danékova Hadile Mutar Sébastien Rouget Xavier Torterüe Elodie Nattes Isabelle Hau Sandra Biscardi Houmam El Jurdi Camille Jung Ralph Epaud Céline Delestrain Adèle Carlier‐Gonod Camille Chavy Benoît Colomb Stéphanie Litzler-Renault Denis S. Semama Frédéric Huet Mayssa Sarakbi Guillaume Mortamet C. Bost-Bru Charlotte Kevorkian‐Verguet Matthias Lachaud Caroline Vinit V. Hentgen Pascal Leroux Valérie Bertrand Caroline Parrod Irina Craiu

<h3>Importance</h3> Multisystem inflammatory syndrome in children (MIS-C) is the most severe pediatric disease associated with acute respiratory coronavirus 2 infection, potentially life-threatening, but optimal therapeutic strategy remains unknown. <h3>Objective</h3> To compare intravenous immunoglobulins (IVIG) plus methylprednisolone vs IVIG alone as initial therapy MIS-C. <h3>Design, Setting, and Participants</h3> Retrospective cohort study drawn from a national surveillance system...

10.1001/jama.2021.0694 article EN JAMA 2021-02-02

Type I interferons (IFNs) are essential mediators of antiviral responses. These cytokines have been implicated in the pathogenesis autoimmunity, most notably systemic lupus erythematosus (SLE), diabetes mellitus, and dermatomyositis, as well monogenic type interferonopathies. Despite a fundamental role health disease, direct quantification IFNs has challenging. Using single-molecule array (Simoa) digital ELISA technology, we recorded attomolar concentrations IFNα healthy donors, viral...

10.1084/jem.20161451 article EN cc-by-nc-sa The Journal of Experimental Medicine 2017-04-18

End of April 2020, French clinicians observed an increase in cases presenting with paediatric inflammatory multisystem syndrome (PIMS). Nationwide surveillance was set up and demonstrated temporospatial association the coronavirus disease (COVID-19) epidemic for 156 reported as at 17 May: 108 were classified confirmed (n = 79), probable 16) or possible 13) post-COVID-19 PIMS cases. A continuum clinical features from Kawasaki-like to myocarditis observed, requiring intensive care 67%

10.2807/1560-7917.es.2020.25.22.2001010 article EN cc-by Eurosurveillance 2020-06-04
Jérôme Avouac Élodie Drumez É. Hachulla Raphaèle Séror Sophie Georgin‐Lavialle and 95 more Soumaya El Mahou Édouard Pertuiset Thao Pham Hubert Marotte Amélie Servettaz F. Domont Pascal Chazerain Mathilde Devaux Pascal Claudepierre Vincent Langlois A. Mékinian A. Maria Béatrice Banneville Bruno Fautrel J. Pouchot Thierry Thomas René‐Marc Flipo Christophe Richez Florence A. Aeschlimann C. Agard Nassim Ait-Abdallah Jean-David Albert Didier Alcais Jean‐Sébastien Allain Yannick Allanore Blanca Amador-Borreiro Zahir Amoura Emma André 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 F. Banal Pierre Barbery Thomas Barnetche A. Barrelet A. Basch Vincent Baumier Guillaume Bayer S. Bayle Catherine Beauvais Rudie Beinat Véronique D. Belin Rakiba Belkhir Ruben Benaïnous Alexandre Bélot Mohamed Ben Ammar Mathilde Benhamou Y. Benhamou Ahmed Benmansour Pascal Bennet Brigitte Bernoux-Manat E. Berthet E. Berthoux Ewa Bertolini Adrien Bigot Aurélia Bisson-Vaivre Thomas Gille G Bolla Olivier Bonidan Christine Bonnet Raphaël Borie Marie Bossert Laurence Boudou Françoise Bouhour Kévin Bouiller B. Bouldoires Karima Boussoualim Éric Bouvard Regine Brondino P. Buchlin Laurence Cabantous P. Cacoub Simon Cadiou Maurizio Carteni Aurélia Carbasse Brice Castel P. Cathébras Hervé Caumont Annalisa Celant Benjamin Chaigne Benoît Chaillous Romuald Champy Agnès Charcot

10.1016/s2665-9913(21)00059-x article EN publisher-specific-oa The Lancet Rheumatology 2021-03-25
Florence A. Aeschlimann Nassim Ait-Abdallah Jean-David Albert Didier Alcais Yannick Allanore and 95 more Blanca Amador-Borreiro Zahir Amoura Emma André Jean‐Benoît Arlet Laurent Arnaud Denis Arniaud Herliette Arty-Hue Lucie Atlan Christine Audoin-Pajot Victor Audren Jérôme Avouac Maxime Bach-Bunner Hélène Bacquet-Deschryver Brigitte Bader‐Meunier Nathalie Balandraud Jean‐Charles Balblanc Stéphane Bally F. Banal Béatrice Banneville Thomas Barnetche A. Barrelet A. Basch Vincent Baumier Guillaume Bayer S. Bayle Véronique D. Belin Rakiba Belkhir Ruben Benaïnous Alexandre Bélot Mohamed Ben Ammar Mathilde Benhamou Y. Benhamou Ahmed Benmansour Pascal Bennet Brigitte Bernoux-Manat E. Berthoux Ewa Bertolini Aurélia Bisson-Vaivre Thomas Gille G Bolla Olivier Bonidan Christine Bonnet Raphaël Borie Laurence Boudou Françoise Bouhour Kévin Bouiller B. Bouldoires Karima Boussoualim Éric Bouvard Regine Brondino P. Buchlin P. Cacoub Maurizio Carteni Aurélia Carbasse Brice Castel P. Cathébras Hervé Caumont Annalisa Celant Benjamin Chaigne Benoît Chaillous Romuald Champy Agnès Charcot Pierre Charles Isabelle Charlot-Lambrecht Emmanuel Chatelus B. Chaudier Pascal Chazerain P Chertok Xavier Chevalier Maxime Chevreau Emilie Chotard Lin Chu Miow Pascal Claudepierre Gaëlle Clavel Cyril Clavel-Osorio Fleur Cohen Gregory H. Cohen Marie-Eve Colette-Cedoz Nived Collercandy Antoine Colombey C. Comarmond Bernard Combe Céline Comparon Élodie Constant Clémence Corre N. Costedoat‐Chalumeau Marie Couret Natacha Courvoisier Fabienne Coury Cécile Coutarel F. Coutier R. Damade Laurence Daver-Malaterre Sarahe Dehimat Michel Delahousse

There is little known about the impact of SARS-CoV-2 on patients with inflammatory rheumatic and musculoskeletal diseases (iRMD). We examined epidemiological characteristics associated severe disease, then death. also compared mortality between hospitalised for COVID-19 without iRMD.Individuals suspected iRMD-COVID-19 were included in this French cohort. Logistic regression models adjusted age sex used to estimate ORs 95% CIs COVID-19. The most significant clinically relevant factors...

10.1136/annrheumdis-2020-218310 article EN cc-by-nc Annals of the Rheumatic Diseases 2020-12-02
Jérémy Manry Paul Bastard Adrian Gervais Tom Le Voyer Jérémie Rosain and 95 more Quentin Philippot Eleftherios Michailidis Hans-Heinrich Hoffmann Shohei Eto Marina García-Prat Lucy Bizien Alba Parra-Martínez Rui Yang Liis Haljasmägi Mélanie Migaud Karita Särekannu Julia Maslovskaja Nicolas de Prost Yacine Tandjaoui-Lambiotte Charles‐Édouard Luyt Blanca Amador-Borrero Alexandre Gaudet Julien Poissy Pascal Morel Pascale Richard Fabrice Cognasse Jesús Troya Sophie Trouillet‐Assant Alexandre Bélot Kahina Saker Pierre Garçon Jacques G. Rivière Jean‐Christophe Lagier Stéphanie Gentile Lindsey B. Rosen Elana Shaw Tomohiro Morio Junko Tanaka David Dalmau Pierre‐Louis Tharaux D. Sène Alain Stépanian Bruno Mégarbane Vasiliki Triantafyllia Arnaud Fekkar James R. Heath José Luis Franco Juan‐Manuel Anaya Jordi Solé‐Violán Luisa Imberti Andrea Biondi Paolo Bonfanti Riccardo Castagnoli Ottavia M. Delmonte Yu Zhang Andrew L. Snow Steven M. Holland Catherine M. Biggs Marcela Moncada‐Vélez Andrés A. Arias Lazaro Lorenzo Soraya Boucherit Dany Anglicheau Anna M. Planas Filomeen Haerynck Sotiriјa Duvlis Tayfun Özçelık Sevgi Keleş Ahmed Aziz Bousfiha Jalila El Bakkouri Carolina Ramírez‐Santana Stéphane Paul Qiang Pan‐Hammarström Lennart Hammarström Annabelle Dupont Alina Kurolap Christine N. Metz Alessandro Aiuti Giorgio Casari Vito Lampasona Fabio Ciceri Lucila Akune Barreiros Elena Domínguez‐Garrido Mateus Vidigal Mayana Zatz Diederik van de Beek Sabina Sahanic Ivan Tancevski Yuriy Stepanovskyy Oksana Boyarchuk Yoko Nukui Miyuki Tsumura Loreto Vidaur Stuart G. Tangye Sonia Burrel Darragh Duffy Lluís Quintana‐Murci Adam Klocperk Nelli Y. Kann Anna Shcherbina

Significance There is growing evidence that preexisting autoantibodies neutralizing type I interferons (IFNs) are strong determinants of life-threatening COVID-19 pneumonia. It important to estimate their quantitative impact on mortality upon SARS-CoV-2 infection, by age and sex, as both the prevalence these risk death increase with higher in men. Using an unvaccinated sample 1,261 deceased patients 34,159 individuals from general population, we found against IFNs strongly increased...

10.1073/pnas.2200413119 article EN cc-by Proceedings of the National Academy of Sciences 2022-05-16

IFN-I and IFN-III immunity in the nasal mucosa is poorly characterized during SARS-CoV-2 infection. We analyze IFN-I/III signature, namely expression of ISGF-3-dependent IFN-stimulated genes, mildly symptomatic COVID-19 patients show its correlation with serum IFN-α2 levels, which peak at symptom onset return to baseline from day 10 onward. Moreover, signature correlates nasopharyngeal viral load associated presence infectious viruses. By contrast, we observe low scores despite high loads a...

10.1084/jem.20211211 article EN cc-by-nc-sa The Journal of Experimental Medicine 2021-08-06
Danyel Lee Jérémie Le Pen Ahmad Yatim Beihua Dong Yann Aquino and 95 more Masato Ogishi Rémi Pescarmona Estelle Talouarn Darawan Rinchai Peng Zhang Magali Perret Zhiyong Liu Iolanda Jordán Şefika Elmas Bozdemir Gülsüm İclal Bayhan Camille Beaufils Lucy Bizien Aurélie Bisiaux Wei‐Te Lei Milena Hasan Jie Chen Christina Gaughan Abhishek Asthana Valentina Libri Joseph M. Luna Fabrice Jaffré Hans-Heinrich Hoffmann Eleftherios Michailidis Marion Moreews Yoann Seeleuthner Kaya Bilgüvar Shrikant Mane Carlos Flores Yu Zhang Andrés A. Arias Rasheed Bailey Agatha Schlüter Baptiste Milisavljevic Benedetta Bigio Tom Le Voyer Marie Materna Adrian Gervais Marcela Moncada‐Vélez Francesca Pala Tomi Lazarov Romain Lévy Anna‐Lena Neehus Jérémie Rosain Jessica N. Peel Yi‐Hao Chan Marie‐Paule Morin Rosa Pino Serkan Belkaya Lazaro Lorenzo Jordi Antón Selket Delafontaine Julie Toubiana Fanny Bajolle Victòria Fumadó Marta L. DeDiego Nadhira Fidouh Flore Rozenberg Jordi Pèrez‐Tur Shuibing Chen Todd Evans Frédéric Geissmann Pierre Lebon Susan R. Weiss Damien Bonnet Xavier Duval Qiang Pan‐Hammarström Anna M. Planas Isabelle Meyts Filomeen Haerynck Aurora Pujol Vanessa Sancho‐Shimizu Clifford L. Dalgard Jacinta Bustamante Anne Puel Stéphanie Boisson‐Dupuis Bertrand Boisson Tom Maniatis Qian Zhang Paul Bastard Luigi D. Notarangelo Vivien Béziat Rebeca Pérez de Diego Carlos Rodríguez‐Gallego Helen C. Su Richard P. Lifton Emmanuelle Jouanguy Aurélie Cobat Laia Alsina Sevgi Keleş Élie Haddad Laurent Abel Alexandre Bélot Lluís Quintana‐Murci Charles M. Rice Robert H. Silverman

Multisystem inflammatory syndrome in children (MIS-C) is a rare and severe condition that follows benign COVID-19. We report autosomal recessive deficiencies of OAS1 , OAS2 or RNASEL five unrelated with MIS-C. The cytosolic double-stranded RNA (dsRNA)–sensing generate 2′-5′-linked oligoadenylates (2-5A) activate the single-stranded RNA–degrading ribonuclease L (RNase L). Monocytic cell lines primary myeloid cells OAS1, OAS2, RNase produce excessive amounts cytokines upon dsRNA acute...

10.1126/science.abo3627 article EN cc-by Science 2022-12-20
Qian Zhang Daniela Matuozzo Jérémie Le Pen Danyel Lee Leen Moens and 95 more Takaki Asano Jonathan Bohlen Zhiyong Liu Marcela Moncada‐Vélez Yasemin Kendir Demirkol Huie Jing Lucy Bizien Astrid Marchal Hassan Abolhassani Selket Delafontaine Giorgia Bucciol Laurent Abel Hassan Abolhassani Alessandro Aiuti Özge Metin Akcan Saleh Al‐Muhsen Fahd Al‐Mulla Gülsüm Alkan Mark S. Anderson Evangelos Andreakos Andrés A. Arias Jalila El Bakkouri Hagit Baris Feldman Alexandre Bélot Catherine M. Biggs Dusan Bogunovic Alexandre Bolze Анастасія Бондаренко Ahmed Aziz Bousfiha Şefika Elmas Bozdemir Petter Brodin Yenan T. Bryceson Carlos D. Bustamante Manish J. Butte Giorgio Casari John Christodoulou Roger Colobrán Antônio Condino‐Neto Stefan N. Constantinescu Megan A. Cooper Clifton L. Dalgard Murkesh Desai Beth A. Drolet Jamila El Baghdadi Melike Emiroğlu Emine Hafize Erdeniz Sara Elva Espinosa‐Padilla Jacques Fellay Carlos Flores José Luis Franco Antoine Froidure Peter K. Gregersen Bodo Grimbacher Belgi̇n Gülhan Filomeen Haerynck David Hagin Rabih Halwani Lennart Hammarström James R. Heath Sarah E. Henrickson Elena W.Y. Hsieh Eystein S. Husebye Kohsuke Imai Yuval Itan Petr Jabandžiev Erich D. Jarvis Timokratis Karamitros Adem Karbuz Kai Kisand Cheng‐Lung Ku YL Lau Yun Ling C. Lucas Tom Maniatis Davood Mansouri László Maródi Ayşe Metìn Isabelle Meyts Joshua D. Milner Kristina Mironska Trine H. Mogensen Tomohiro Morio Lisa F. P. Ng Luigi D. Notarangelo Antonio Novelli Giuseppe Novelli Cliona OʼFarrelly Satoshi Okada Keisuke Okamoto Şadiye Kübra Tüter Öz Tayfun Özçelık Qiang Pan‐Hammarström Maria Papadaki Jean W. Pape Aslınur Özkaya Parlakay

Recessive or dominant inborn errors of type I interferon (IFN) immunity can underlie critical COVID-19 pneumonia in unvaccinated adults. The risk children, which is much lower than adults, remains unexplained. In an international cohort 112 children (&amp;lt;16 yr old) hospitalized for pneumonia, we report 12 (10.7%) aged 1.5–13 with (7 children), severe (3), and moderate (2) 4 the 15 known clinically recessive biochemically complete IFN immunity: X-linked TLR7 deficiency children) autosomal...

10.1084/jem.20220131 article EN cc-by The Journal of Experimental Medicine 2022-06-16
Frank B. Cortazar John L. Niles David Jayne Peter A. Merkel Annette Bruchfeld and 95 more Huibin Yue Thomas J. Schall Pirow Bekker Chen Au Peh Aron Chakera Bruce A. Cooper Jadadeesh Kurtkoti Daman Langguth Vicki Levidiotis G. Luxton Peter F. Mount David W. Mudge Euan Noble R. Phoon Dwarakanathan Ranganathan A. Ritchie Jessica Ryan M. Suranyi Alexander R. Rosenkranz K. Lhotta Andreas Kronbichler Nathalie Demoulin C. Bovy R. Hellemans Jean‐Michel Hougardy Ben Sprangers Katherine D. Wissing Christian Pagnoux S. Barbour Soumeya Brachemi S. Cournoyer Louis Girard Louis‐Philippe Laurin Patrick Liang David Philibert Michael Walsh Vladimı́r Tesař Radim Bečvář P. Horak Ivan Rychlík Wladimir Szpirt Hans Dieperink Jon Waarst Gregersen Per Ivarsen Elizabeth Krarup Cecilie Lyngsoe Claire Rigothier Jean‐François Augusto Alexandre Bélot Dominique Chauveau Divi Cornec Noémie Jourde‐Chiche M. Ficheux Alexandre Karras Armelle Klein F. Maurier Rafik Mesbah Olivier Moranne A. Néel T. Quéméneur David Saadoun Benjamin Terrier Philippe Zaoui Matthias Schaier Urs Benck Raoul Bergner Matthias H. Busch Jürgen Floege Franziska Grundmann Hermann Haller Marion Haubitz B. Hellmich Jörg Henes Bernd Hohenstein C. Hugo Christof Iking‐Konert Fabian Arndt Torsten Kubacki Ina Kötter Peter Lamprecht Tom H. Lindner Jan Halbritter H. Mehling Ulf Schönermarck Nils Venhoff Volker Vielhauer Oliver Witzke István Szombati G Szücs Giacomo Garibotto Federico Alberici Enrico Brunetta Lorenzo Dagna Salvatore De Vita Giacomo Emmi

In the 330-patient ADVOCATE trial of avacopan for treatment antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, in which 81% patients had renal involvement, estimated glomerular filtration rate (eGFR) increased on average 7.3 ml/min per 1.73 m2 group and 4.1 prednisone (P = 0.029) at week 52. This new analysis examines results patient subgroup with severe insufficiency enrollment into trial, i.e., eGFR ≤20 m2.eGFR was determined baseline over course trial. Changes were...

10.1016/j.ekir.2023.01.039 article EN cc-by-nc-nd Kidney International Reports 2023-02-03

Type I and III interferons (IFN-I/λ) are important antiviral mediators against SARS-CoV-2 infection. Here, we demonstrate that plasmacytoid dendritic cells (pDC) the predominant IFN-I/λ source following their sensing of SARS-CoV-2-infected cells. Mechanistically, this short-range by pDCs requires sustained integrin-mediated cell adhesion with infected In turn, restrict viral spread an response directed toward This specialized function enables to efficiently turn-off replication, likely via a...

10.1038/s41467-023-36140-9 article EN cc-by Nature Communications 2023-02-08
Paul Bastard Adrian Gervais Maki Taniguchi Liisa Saare Karita Särekannu and 95 more Tom Le Voyer Quentin Philippot Jérémie Rosain Lucy Bizien Takaki Asano Marina García-Prat Alba Parra-Martínez Mélanie Migaud Miyuki Tsumura Francesca Conti Alexandre Bélot Jacques G. Rivière Tomohiro Morio Junko Tanaka Étienne Javouhey Filomeen Haerynck Sotiriјa Duvlis Tayfun Özçelık Sevgi Keleş Yacine Tandjaoui-Lambiotte Simon Escoda M. Husain Qiang Pan‐Hammarström Lennart Hammarström Gloria Ahlijah A Haidar Camille Soudée Vincent Arseguel Hassan Abolhassani Sabina Sahanic Ivan Tancevski Yoko Nukui Seiichi Hayakawa George P. Chrousos Athanasios Michos Elizabeth‐Barbara Tatsi Filippos Filippatos Agustí Rodríguez‐Palmero Jesús Troya Imran Tipu Isabelle Meyts Lucie Roussel Sisse Rye Ostrowski Laire Schidlowski Carolina Prando Antônio Condino‐Neto Nathalie Cheikh Ahmed Aziz Bousfiha Jalila El Bakkouri Sergio Aguilera Suzan A. AlKhater Gülsüm Alkan Riccardo Castagnoli Cyril Cyrus Şefika Elmas Bozdemir Melike Emiroğlu Belgi̇n Gülhan Emine Hafize Erdeniz Nevin Hatipoğlu Gülsün Iclal Bayhan Petr Jabandžiev Saliha Kanık Yüksek Adem Karbuz Şadiye Kübra Tüter Öz Gian Luigi Marseglia Özge Metin Akcan Ahmet Osman Kılıç Aslınur Özkaya Parlakay Maria Papadaki Kateřina Slabá Esra Şevketoğlu Juan Valencia-Ramos Aysun Yahşi Antonio Aguilera J. Álvarez Antonio Antela López Gema Barbeito Castiñeiras Xabier Bello Paderne Miriam Ben García María Victoria Carral García Miriam Cebey‐López Amparo Coira M. Pájaro José Javier Costa Alcalde María José Currás Tuala Ana Isabel Dacosta Urbieta Blanca Díaz Esteban María Jesús Domínguez Santalla Cristina Fernández Juan Fernández Villaverde Cristóbal Galbán Rodríguez José Luis García Allut Luisa García Vicente Elena Giráldez Vázquez Alberto Gómez‐Carballa

We found that 19 (10.4%) of 183 unvaccinated children hospitalized for COVID-19 pneumonia had autoantibodies (auto-Abs) neutralizing type I IFNs (IFN-α2 in 10 patients: IFN-α2 only three, plus IFN-ω five, and IFN-α2, IFN-β two; nine patients). Seven (3.8%) Abs at least ng/ml one IFN, whereas the other 12 (6.6%) 100 pg/ml. The auto-Abs neutralized both unglycosylated glycosylated IFNs. also detected pg/ml 4 2,267 uninfected (0.2%) 45 (2%). odds ratios (ORs) life-threatening were, therefore,...

10.1084/jem.20231353 article EN cc-by The Journal of Experimental Medicine 2024-01-04

Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis management is still missing. In May 2022, EULAR PReS endorsed proposal joint task force (TF) to develop recommendations of sJIA AOSD. The TF agreed during first meeting address four topics: similarity between AOSD, diagnostic biomarkers, therapeutic targets strategies complications including macrophage activation syndrome (MAS). Systematic...

10.1136/ard-2024-225851 article EN Annals of the Rheumatic Diseases 2024-09-24
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