Abstracts tagged "complement"

Abstract Number: 0608 • ACR Convergence 2024
Systematic Analysis Demonstrates the Added Value of CB-CAPs to SLE Diagnosis in a Large Validation Cohort
Andrew Concoff¹, Touba Warsi², Sepehr Taghavi², Sudha Kumar², Abigail Patalinghug², Christine Schleif², Brittany Partain³, Joseph Ahearn⁴, Chau-Ching Liu⁵, Nicole Wilson⁵, Susan Manzi⁵ and Tyler O'Malley⁶, ¹Exagen, Inc., Los Angeles, CA, ²Exagen, Carlsbad, CA, ³Exagen, Boston, MA, ⁴Allegheny Health Network, Wexford, PA, ⁵Allegheny Health Network, Pittsburgh, PA, ⁶Exagen, Vista, CA
Background/Purpose: Cell bound complement activation products (CB-CAPs) including erythrocyte-bound C4d (EC4d) and B-lymphocyte C4d (BC4d) demonstrate increased diagnostic accuracy compared to conventional SLE markers (anti-dsDNA,…
Abstract Number: 2534 • ACR Convergence 2024
Deciphering Complement-dependent Macrophage Phenotypes in Human Rheumatoid Arthritis Using Combined Computational-experimental Single-cell Omics
Juan Vargas¹, Ian Mantel², Jun Inamo³, Nirmal Banda¹, Anna Helena Jonsson³, Kevin Wei⁴, Deepak Rao⁵, Susan Goodman⁶, Kevin Deane⁷, Jennifer Seifert⁸, Jennifer Anolik⁹, Michael Brenner¹⁰, Soumya Raychaudhuri¹¹, Trent Woodruff¹², the Accelerating Medicines Partnership (AMP) RA/SLE Network¹³, Michael Holers¹⁴, Laura Donlin¹⁵ and Fan Zhang¹⁶, ¹The University of Colorado, Aurora, CO, ²WEILL CORNELL MEDICINE, New York, NY, ³University of Colorado School of Medicine, Aurora, CO, ⁴Brigham and Women's Hospital at Harvard Medical School, Boston, MA, ⁵Brigham and Women's Hospital, Harvard Medical School, Boston, MA, ⁶Hospital for Special Surgery, New York 10025, NY, ⁷University of Colorado Denver Anschutz Medical Campus, Aurora, CO, ⁸University of Colorado and Oklahoma Medical Research Foundation, Aurora, CO, ⁹University of Rochester Medical Center, Rochester, NY, ¹⁰Brigham Women's Hospital, Boston, MA, ¹¹Brigham and Women's Hospital, Boston, MA, ¹²University of Queensland, Brisbane, Australia, ¹³AMP RA/SLE consortium, Aurora, CO, ¹⁴Division of Rheumatology, University of Colorado School of Medicine, Aurora, CO, ¹⁵Hospital for Special Surgery, New York, NY, ¹⁶University of Colorado, Aurora, CO
Background/Purpose: The complement system is a major component of innate immunity and plays a vital role in experimental models of autoimmune disease pathogenesis. In patients…
Abstract Number: 0617 • ACR Convergence 2024
Hereditary C1q Deficiency Is Associated with Type 1 Interferon-pathway Activation and a High Risk of Central Nervous System Inflammation
Clément triaille¹, Neha Mohan Rao², Gillian Rice³, Luis Seabra⁴, Sutherland Fraser⁵, Vincent Bondet⁶, Darragh duffy⁶, Andrew Gennery⁷, Benjamin Fournier⁸, Brigitte Bader-Meunier⁹, Christopher Troedson¹⁰, Gavin CLeary¹¹, Helena Buso¹², Jacqueline Dalby-Payne¹³, Ranade Prajakta¹⁴, Katrien Jansen¹⁵, Lien De Somer¹⁶, Marie-Louise Fremond¹⁷, Pimple Pallavi¹⁴, Melanie Wong¹⁸, Russel Dale¹⁰, Carine Wouters¹⁶, PIERRE QUARTIER¹⁹, Raju Khubchandani²⁰ and Yanick Crow⁵, ¹Pôle de pathologies rhumatismales systémiques et inflammatoires, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium, Brussels, Belgium, ²NH SRCC hospital, Department of Pediatric Rheumatology, Mumbai, Maharashtra, India, Mumbai, ³Division of Evolution and Genomic Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester Academic Health Science Centre, Manchester, United Kingdom, Manchester, ⁴Laboratory of Neurogenetics and Neuroinflammation, Imagine Institute, INSERM UMR1163, Paris, France, Paris, France, ⁵MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, Edinburgh, United Kingdom, Edinburgh, United Kingdom, ⁶Translational Immunology Unit, Institut Pasteur, Université Paris-Cité, Paris, France, Paris, France, ⁷: Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United Kingdom, Newcastle, United Kingdom, ⁸Paediatric Immunology-Hematology and Rheumatology Unit, Necker Hospital, APHP Centre, Université Paris-Cité, Paris, France, Paris, France, ⁹Necker Hospital, Assistance Publique Hopitaux de Paris, Paris, France, ¹⁰T. Y. Nelson Department of Neurology and Neurosurgery, Children's Hospital at Westmead, Westmead, New South Wales, and University of Sydney, Australia, Sydney, Australia, ¹¹: Paediatric Rheumatology, Alder Hey Children's Hospital, Liverpool, United Kingdom, Liverpool, United Kingdom, ¹²Department of Medicine - DIMED, University of Padova, Padova, Italy, Padova, Italy, ¹³Specialty of Child and Adolescent Health, Faculty of Medicine, The University of Sydney, Australia, Sydney, Australia, ¹⁴NH SRCC hospital, Department of Pediatric Rheumatology, Mumbai, Maharashtra, India, Mumbai, India, ¹⁵Division of Pediatric Neurology, Department of Pediatrics, University Hospitals Leuven, Belgium, Leuven, Belgium, ¹⁶Division of Pediatric Rheumatology, Department of Pediatrics, University Hospitals Leuven, Belgium, Leuven, Belgium, ¹⁷Paediatric Immunology-Hematology and Rheumatology Unit, Necker Hospital, APHP Centre, Université Paris-Cité, Paris, France, Paris, United Kingdom, ¹⁸Department of Allergy and Immunology, Children's Hospital at Westmead, Westmead, Australia, Westmead, Australia, ¹⁹Université Paris-Cite, IMAGINE Institute, Necker Children’s Hospital, Paris Cedex 15, France, ²⁰SRCC Childrens Hospital, Mumbai, India
Background/Purpose: To report on the largest cohort of C1Q deficient (C1QDef ) patients; to investigate the activation of Type 1 interferon pathway in the blood…
Abstract Number: 0629 • ACR Convergence 2024
Evaluating the Concordance Between SRI4 and BICLA Using Placebo Data from Randomized Controlled Trials of Patients with Active Systemic Lupus Erythematosus
Anca Askanase¹, Edward Vital², Oliver Meier³, Armando Turchetta⁴, Huiyan (Ashley) Mao⁴, Justine Maller⁵, Jorge A. Ross Terres⁶ and Maria Dall'Era⁷, ¹Columbia University Medical Center, New York, NY, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health, University of Leeds, Leeds, United Kingdom, Leeds, England, United Kingdom, ³F. Hoffmann-La Roche Ltd, Basel, Switzerland, Basel, Switzerland, ⁴Hoffmann-La Roche Ltd, Mississauga, Canada, ⁵Genentech, Inc,, San Francisco, CA, ⁶Genentech, Inc,, San Francisco, ⁷UCSF, Corte Madera, CA
Background/Purpose: British Isles Lupus Assessment Group (BILAG)-based Composite Lupus Assessment (BICLA) and Systemic Lupus Erythematosus Responder Index 4 (SRI4) responses are the most common primary…
Abstract Number: 0669 • ACR Convergence 2024
Efficacy of Belimumab in Systemic Lupus Erythematosus: A Single-Centre Retrospective Study
buddhika Jayaratna¹, Pasan Serasinghe², Louise Nel³, Sahil Jain³, Shrish Sangle², Begona Lopez¹, Giovanni Sanna², Michelle Fernando¹ and David DCruz⁴, ¹Guys & St Thomas NHS Foundation Trust, London, United Kingdom, ²Guys & St Thomas NHS Foundation Trust, London, England, United Kingdom, ³Guys and St Thomas' Hospital, London, United Kingdom, ⁴The Louise Coote Lupus Unit, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, United Kingdom
Background/Purpose: Systemic Lupus Erythematosus (SLE) is a chronic multi-system autoimmune rheumatic disorder. Despite therapeutic advances, managing SLE remains challenging due to its complex pathogenesis and…
Abstract Number: 1378 • ACR Convergence 2024
Tocilizumab Effect on the Three Pathways of the Complement System in Patients with Rheumatoid Arthritis
Marta Hernández-Díaz¹, Iván Ferraz-Amaro², Sergio Santos-Concepción³, Javier Castro³, Vanesa Hernandez⁴, Beatriz Tejera Segura⁵, Cristina Luna⁶, Esmeralda Delgado-Frías² and Federico Diaz-González², ¹Hospital Universitario de Canarias, SANTA CRUZ DE TENERIFE, Spain, ²Hospital Universitario de Canarias, La Laguna, Spain, ³Universidad de La Laguna, La Laguna, Spain, ⁴Rheumatology department. Hospital Universitario de Canarias, La Laguna, Spain, ⁵Rheumatology Department. Hospital Universitario Insular de Gran Canaria, Las palmas, Spain, ⁶Hospital Universitario Nuestra Señora de La Candelaria, Santa Cruz, Spain
Background/Purpose: Tocilizumab (TCZ) is used in diseases characterized by markedly elevated inflammatory markers and elevated pro-inflammatory cytokines like rheumatoid arthritis (RA), juvenile idiopathic arthritis and…
Abstract Number: 1490 • ACR Convergence 2024
Multi-centered Clinical Validation of T Cell-bound C4d (TC4d) and T Cell Autoantibodies (TIgG and TIgM): Sensitive and Specific Biomarkers of SLE with Enhanced Accuracy Compared to Conventional SLE Tests
Vasileios Kyttaris¹, Andrew Concoff², Touba Warsi³, Sepehr Taghavi³, Sudha Kumar³, Stanley Park³, Abigail Patalinghug³, Christine Schleif³, Brittany Partain⁴, Joseph Ahearn⁵, Nicole Wilson⁶, Chau-Ching Liu⁶, Susan Manzi⁶ and Tyler O'Malley⁷, ¹BIDMC, Boston, MA, ²Exagen, Inc., Los Angeles, CA, ³Exagen, Carlsbad, CA, ⁴Exagen, Boston, MA, ⁵Allegheny Health Network, Wexford, PA, ⁶Allegheny Health Network, Pittsburgh, PA, ⁷Exagen, Vista, CA
Background/Purpose: Conventional SLE diagnostic markers lack sensitivity and are biased towards severe disease, resulting in a subset of clinically ambiguous ANA-positive but specific autoantibody-negative patients.…
Abstract Number: 1607 • ACR Convergence 2024
Low Concentrations of Anti-C5a Complement Receptor Antibodies Are Associated with Relapse in Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Sebastian Klapa¹, Sabrina Arnold², Antje Müller², Andreas Koch³, Anja Staehle⁴, Wataru Kähler³, Harry Heidecke⁵, Gabriela Riemekasten² and Peter Lamprecht², ¹University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, ²University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, Germany, ³Christian-Albrechts-University Kiel, Institute of Experimental Medicine, Kiel, Germany, ⁴University of Lübeck, Department of Rheumatology and Immunology, Lübeck, Germany, ⁵CellTrend GmbH, Luckenwalde, Germany
Background/Purpose: Complement activation has been shown to play an important role in the pathogenesis of the two major anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) variants,…
Abstract Number: 1703 • ACR Convergence 2024
Single Cell and Spatial Transcriptomics Implicate Vascular Cell Orchestration of Inflammatory Changes After Ultraviolet Light Exposure
Rachael Wasikowski¹, Erin Theisen², Jie An³, Lam C. Tsoi¹, Ksenia Anufrieva⁴, Kevin Wei⁵, Johann Gudjonsson⁶ and Keith Elkon³, ¹Michigan, Dept. of Dermatology, Ann Arbor, MI, ²Brigham and Women’s Hospital and Harvard Medical School, Boston, MA, ³University of Washington, Seattle, WA, ⁴BWH, Cambridge, MA, ⁵Brigham and Women's Hospital at Harvard Medical School, Boston, MA, ⁶University of Michigan, Ann Arbor, MI
Background/Purpose: Skin sensitivity to sunlight affects ~70% of systemic lupus erythematosus (SLE). In addition, ultraviolet radiation (UVR) can stimulate systemic disease flares, but how this…
Abstract Number: 2065 • ACR Convergence 2024
Proposed Pathways Involved in the Pathogenesis of Juvenile Dermatomyositis (JDM)
Samantha Coss¹, Danlei Zhou¹, Rabheh Abdul Aziz², Katherine Miller³, Shoghik Akoghlanian⁴, Kyla Driest¹, Edward Oberle¹, Vidya Sivaraman⁵, Charles Spencer⁶, Stacy Ardoin¹ and Chack-Yung Yu⁷, ¹Nationwide Children's Hospital, Columbus, OH, ²University at Buffalo, Buffalo, NY, ³Nationwide Children's Hospital, Institute for Genomic Medicine, Columbus, OH, ⁴Nationwide Children�s Hospital, Columbus, OH, ⁵Nationwide Children's Hospital/ The Ohio State University, Columbus, OH, ⁶University of Mississippi Medical Center, Jackson, MS, ⁷Abigail Wexner Research Institute at Nationwide Children's Hospital, Columbus, Ohio, OH
Background/Purpose: JDM is an autoimmune disease of skin and muscle. We investigated genetic factors, autoantibodies, and clinical features in JDM.Methods: Subjects came from 2 large…
Abstract Number: 0040 • ACR Convergence 2024
Targeting Complement Factor B (CFB) via a Novel siRNA Therapy (AZD6912) to Treat Inflammatory Arthritis
Gary Sims¹, Mia Collins², Natalie Pursell³, Teneema Kuriakose¹, Fanyi Jiang⁴, Kristi Moore⁵, Lucie Bertrand⁵, Melissa Lasaro⁵, Jessica Neisen⁶, Scott Manetz⁷, Gabriel Wong⁸, Patrick Riley⁵, Iain McInnes⁹, Kyriakos Konstantinidis¹⁰ and David Close⁸, ¹Immunology Biosciences, Research and Early Development, Respiratory and Immunology (R&I), BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD, ²Translational Science and Experimental Medicine, Research and Early Development, Respiratory and Immunology (R&I), BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden, ³Dicerna, Lexington, ⁴Translation and Experimental Medicine, Research and Early Development, Respiratory and Immunology (R&I), BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ⁵Alexion, AstraZeneca Rare Disease, New Haven, CT, ⁶Translational Science and Experimental Medicine, Research and Early Development, Respiratory and Immunology (R&I), BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ⁷Clinical Pharmacology and Safety Science, Research and Early Development, Respiratory and Immunology (R&I), BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD, ⁸Early Clinical Development, Research and Early Development, Respiratory and Immunology (R&I), BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ⁹University of Glasgow, College of Medical Veterinary and Life Sciences, Glasgow, United Kingdom, ¹⁰Projects, Research and Early Development, Respiratory and Immunology (R&I), BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom
Background/Purpose: New precision medicine guided therapies targeting alternate disease driving processes are required to improve clinical outcomes in RA. The complement pathway has long been…
Abstract Number: 2177 • ACR Convergence 2024
CD59 Complement Membrane Regulator Deficiency: 12 Years of Clinical and Molecular Follow-up of 29 Patients
Dror Mevorach, Netanel Karbian, Marian Zeibak and Adi Tabib, Hadassah-University Hospital, Jerusalem, Israel
Background/Purpose: We have described in 2013, homozygous CD59-deficient children that manifest with recurrent peripheral neuropathy resembling Guillain-Barré syndrome (GBS), with hemolytic anemia and recurrent strokes.…
Abstract Number: 0045 • ACR Convergence 2024
Proteomic Analysis of the Rheumatoid Arthritis Citrullinome Reveals an Enrichment of Citrullinated Complement Proteins
Khushali Trivedi¹, Clarissa Klenke², Jun Kim¹, Jeba Atkia Maisha², Alina Sememenko¹, XIAOBO MENG², Mario Navarrete¹, Hani El-Gabalawy² and Liam O'Neil², ¹University of Manitoba, Winnipeg, Canada, ²University of Manitoba, Winnipeg, MB, Canada
Background/Purpose: Citrullination is a key physiological process that drives autoantibody formation in Rheumatoid Arthritis (RA). This irreversible post-translational modification of the amino acid arginine is…
Abstract Number: 2230 • ACR Convergence 2024
Relationship Between the Complement System and Serum Lipid Profile in Patients with Rheumatoid Arthritis
Maria Garcia-Gonzalez¹, Fuensanta Gomez-Bernal², Juan Carlos Quevedo-Abeledo³, Miguel Angel Gonzalez-Gay⁴ and ivan Ferraz-Amaro⁵, ¹Hospital Universitario de Canarias, SC Tenerife, Canarias, Spain, ²Hospital Universitario de Canarias, Santa Cruz de Tenerife, Canarias, Spain, ³Hospital Universitario de Gran Canaria Dr Negrín, Las Palmas de GC, Canarias, Spain, ⁴University of Cantabria, Fundación Jimenez Díaz, Madrid, Madrid, Spain, ⁵Rheumatology, Hospital Universitario de Canarias, Santa Cruz de Tenerife, Canarias, Spain
Background/Purpose: The complement system has been linked to the etiopathogenesis of rheumatoid arthritis (RA). Patients with RA exhibit a dysregulated profile of lipid molecules, which…
Abstract Number: 0114 • ACR Convergence 2024
Rare Germline Variants in Complement Regulatory Genes in Antiphospholipid Antibody Positive Patients: Prospective Results from AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (“Registry”)
Cécile Yelnik¹, shruti chaturvedi², Julien Labreuche³, Xiang-Zuo Pan², H Michael Belmont⁴, Nina Kello⁵, Paul Fortin⁶, David Branch⁷, Yu Zuo⁸, Rohan Willis⁹, Robert Brodsky², Jane Salmon¹⁰, Maria Laura Bertolaccini¹¹, Hannah Cohen¹², Michelle Petri¹³ and Doruk Erkan¹⁰, and on behalf of APS ACTION, ¹lille university, Lille, France, ²John Hopkins University, Baltimore, MD, ³Lille University Hospital, Lille, France, ⁴NYU Langone Health, New York, NY, ⁵Northwell Health, Brooklyn, NY, ⁶Centre ARThrite - CHU de Québec - Université Laval, Quebec, QC, Canada, ⁷University of Utah and Intermountain Healthcare, Salt Lake City, UT, ⁸University of Michigan, Ann Arbor, MI, ⁹University of Texas Medical Branch, Galveston, TX, ¹⁰Hospital for Special Surgery, New York, NY, ¹¹King's College London, London, United Kingdom, ¹²University College London Hospitals NHS Foundation Trust, London, United Kingdom, ¹³Johns Hopkins University School of Medicine, Timonium, MD
Background/Purpose: We previously reported that markers of complement activation, specifically elevated C4d levels and positive modified HAM (mHAM) test, are associated with a higher risk…

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