Abstracts tagged "Inflammation"

Abstract Number: 0133 • ACR Convergence 2022
Retention Rate of Targeted Therapies in Erdheim-Chester Disease
Corrado Campochiaro¹, alessandro tomelleri¹, Francesco Pegoraro², Giovanni Galardi³, Nicola Farina⁴, Martina Mazzariol⁵, Francesco Catamerò⁶, Giacomo De Luca⁴, Giulio Cavalli⁴, Augusto Vaglio⁷ and Lorenzo Dagna⁸, ¹IRCCS San Raffaele Hospital. Vita-Salute San Raffaele University, Milano, Italy, ²Department of Health Sciences, University of Florence, Firenze, Italy, ³Department of Health Sciences, University of Florence, Florence, Italy, ⁴IRCCS San Raffaele Hospital - Vita-Salute San Raffaele University, Milano, Italy, ⁵University of Turin, Torino, Italy, ⁶Vita-Salute San Raffaele University, Milano, Italy, ⁷Azienda Ospedaliero Universitaria Meyer, Parna, Italy, ⁸IRCCS San Raffaele Hospital and Vita-Salute San Raffaele University, Milano, Italy
Background/Purpose: Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis with a broad clinical spectrum. The therapeutic landscape of ECD has remarkably changed over the last…
Abstract Number: 0557 • ACR Convergence 2022
VEXAS Syndrome Is Characterized by Blood and Tissues Inflammasome Pathway Activation and Monocyte Dysregulation
Benjamin Terrier¹, Celine Posseme², Marie Temple³, Aurélien Corneau⁴, Francesco Carbone⁵, Eugénie Duroyon³, camille Gobeaux³, Estibaliz Lazaro⁶, Roderau Outh⁷, Guillaume Le Guenno⁸, Francois Lifermann⁹, Marie Berleur¹⁰, Thierry Weitten¹¹, Vivien Guillotin¹², Cédric Lenormand¹³, Hang-Korng Ea¹⁴, Mickael Ménager⁵, Darragh Duffy² and Olivier Kosmider³, ¹National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ²Institut Pasteur, Paris, France, ³Cochin Hospital, Paris, France, ⁴Pitié Salpêtrière Hospital, Paris, France, ⁵Institut Imagine, Paris, France, ⁶Bordeaux Hospital University, Bordeaux, France, ⁷CH, Perpignan, France, ⁸CHU, Clermont-Ferrand, France, ⁹Dax Hospital, Dax, France, ¹⁰CH, Versailles, France, ¹¹CHIGAS, Gap, France, ¹²CHU Bordeaux, Bordeaux, France, ¹³CHRU, Strasbourg, France, ¹⁴Lariboisière Hospital, Paris, France
Background/Purpose: Acquired mutations in the UBA1 gene, occurring in myeloid cells and resulting in expression of a catalytically impaired isoform of the enzyme E1, were…
Abstract Number: 0693 • ACR Convergence 2022
Low Neutrophil Ectonucleotidase Activity Drives Neutrophil-Platelet Aggregation and Platelet Activation in Antiphospholipid Syndrome (APS)
Naveen Kumar S K¹, Claire Hoy¹, Srilakshmi Yalavarthi¹, Cyrus Sarosh², Kaitlyn Sabb¹, Christine Rysenga¹, Jacqueline Madison¹, Yu Zuo¹ and Jason S Knight³, ¹University of Michigan, Ann Arbor, MI, ²University of Michigan, Temperance, MI, ³University of Michigan, Division of Rheumatology, Ann Arbor, MI
Background/Purpose: By converting extracellular ATP into homeostatic adenosine, the ectonucleotidases CD39 (ATP to AMP) and CD73 (AMP to adenosine) dictate extracellular purinergic gradients and as…
Abstract Number: 1154 • ACR Convergence 2022
Axial Spondyloarthritis: Profiling of MiRNAs in Peripheral Blood Mononuclear Cells
Aneta Pekčcová¹, Kristýna Bubová², Monika Gregova³, Šárka Forejtová², Jana Hořínková², Markéta Hušáková², Michal Tomcik⁴, Jindřiška Gatterová², Jiří Vencovský⁵, Karel Pavelka⁶, Ladislav Šenolt⁵ and Jiří Baloun², ¹Institue of Rheumatology, Prague, Prague, Czech Republic, ²Institue of Rheumatology, Prague, Czech Republic, ³Institue of Rheumatology, Prague, ⁴Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ⁵Institute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic, ⁶Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Praha, Czech Republic
Background/Purpose: microRNAs (miRNAs) are small non-coding RNAs, which are essential for correct gene expression. The dysregulation in miRNA expression is accompanied by various diseases and…
Abstract Number: 1614 • ACR Convergence 2022
Females with Non-Radiographic but Not with Radiographic Axial Spondyloarthritis Are Less Likely to Achieve Inactive Disease State Than Males: Results from a Multicountry Prospective Observational Study
Denis Poddubnyy¹, Joachim Sieper², Servet Akar³, Santiago Muñoz-Fernández⁴, Hildrun Haibel², Torsten Diekhoff⁵, Mikhail Protopopov⁶, Elisabeth Altmaier⁷, Fabiana Ganz⁸ and Robert Inman⁹, ¹Department of Gastroenterology, Infectious Diseases and Rheumatology, Charité – Universitätsmedizin Berlin, Berlin, Germany, ²Charité - Universitätsmedizin, Berlin, Berlin, Germany, ³Izmir Katip Celebi University School of Medicine, Izmir, Turkey, ⁴Servicio de Reumatología, Hospital Universitario Infanta Sofía, Universidad Europea, Madrid, Spain, ⁵Department of Radiology, Charité-Universitätsmedizin Berlin, Berlin, Germany, ⁶Charité Universitätsmedizin Berlin, Berlin, Germany, ⁷GKM Gesellschaft für Therapieforschung mbH, Munich, Germany, ⁸AbbVie, Inc., Luzern, Switzerland, ⁹University Health Network, Toronto, ON, Canada
Background/Purpose: Emerging evidence suggests that men and women experience axial spondyloarthritis (axSpA) differently. Some studies suggested that the probability of treatment response was lower in…
Abstract Number: 1846 • ACR Convergence 2022
Cardiac Sarcoidosis: An Underdiagnosed, Life-threatening yet Treatable Disease
Patrick Webster¹, Yogita Rochlani² and Konstantinos Loupasakis³, ¹Rheumatology Fellow at Montefiore, New York, NY, ²Montefiore Medical Center / Albert Einstein College of Medicine, Bronx, NY, ³Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY
Background/Purpose: Cardiac sarcoidosis (CS) is potentially life-threatening and it typically causes heart failure, ventricular arrhythmia, AV block or sudden death. CS has been reported to…
Abstract Number: 2161 • ACR Convergence 2022
Alterations of Nutritional Status of Scleroderma Patients and Associations with Disease-specific Features
Sabína Oreská¹, Hana Storkanova¹, Maja Spiritovic², Barbora Hermankova², Michal Vrablík³, Karel Pavelka⁴, Jiří Vencovský⁵, Ladislav Šenolt⁵, Radim Becvar¹ and Michal Tomcik¹, ¹Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ²Institute of Rheumatology, Department of Physiotherapy, Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic, Prague, Czech Republic, ³3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ⁴Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Praha, Czech Republic, ⁵Institute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic
Background/Purpose: Systemic sclerosis (SSc) is characterized by skin and organ involvement and chronic disease course. In particular, involvement of the gastrointestinal tract and systemic inflammation…
Abstract Number: 0137 • ACR Convergence 2022
Polyarticular Arthritis and Osteolysis Caused by Mutations in the ASAH1 Gene: Farber Disease Clinical Presentations in the First-ever Natural History Study
Alexander Solyom¹, Kathleen Crosby², Nils Confer² and Jaime Lopez Valdez³, ¹Aceragen, Basel, Switzerland, ²Aceragen, Durham, NC, ³Centenario Hospital Miguel Hidalgo, Aguascalientes, Mexico
Background/Purpose: Farber disease is frequently misdiagnosed as polyarticular juvenile idiopathic arthritis or seronegative rheumatoid arthritis which leads to a delay in diagnosis for many patients.…
Abstract Number: 0561 • ACR Convergence 2022
CD8 T-Cell Expansion May Compensate for Natural Killer Cell Dysfunction in Mice with Chronic Excess IL-18
Jemy Varghese¹, Emily Landy², Scott Canna³, Vinh Dang³ and Paul Tsoukas⁴, ¹University of Pittsburgh, Philadelphia, PA, ²University of Pittsburgh, Pittsburgh, PA, ³Children's Hospital of Philadelphia, Philadelphia, PA, ⁴The Hospital for Sick Children, Toronto, ON, Canada
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) and Macrophage Activation Syndrome (MAS) are associated with both low peripheral Natural Killer (NK) cell numbers and NK dysfunction.…
Abstract Number: 0740 • ACR Convergence 2022
Real-World Demographics, Clinical Characteristics, and Treatment Patterns of Patients Treated with Emapalumab for Secondary Hemophagocytic Lymphohistiocytosis in the United States: The REAL-HLH Study
Carl Allen¹, Shanmuganathan Chandrakasan², Michael Jordan³, Jennifer Leiding⁴, Abiola Oladapo⁵, Priti Pednekar⁶, Kelly Walkovich⁷ and John Yee⁸, ¹Baylor College of Medicine, Houston, TX, ²Emory University School of Medicine, Atlanta, GA, ³University of Cincinnati, Cincinnati, OH, ⁴Johns Hopkins University, Baltimore, MD, ⁵Swedish Orphan Biovitrum AB, Boston, MA, ⁶PRECISIONheor, Los Angeles, CA, ⁷University of Michigan Health, Ann Arbor, MI, ⁸Sobi - North America, Waltham, MA
Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening, hyperinflammatory syndrome caused by overproduction of proinflammatory cytokines, e.g., interferon gamma (IFNγ). Secondary HLH (sHLH), a subtype…
Abstract Number: 1158 • ACR Convergence 2022
Differential Plasma Levels of S100 Proteins in Male versus Female Patients with Ankylosing Spondylitis
Chan Mi Lee¹, Maricela Haghiac² and Marina Magrey³, ¹University Hospitals, Cleveland, OH, ²Metrohealth Medical Center, Cleveland, OH, ³Case Western Reserve University, University Hospitals, Richfield, OH
Background/Purpose: S100 proteins are a family of 25 calcium-binding cytosolic proteins which are involved in stress and inflammatory responses. S100A4 acts as a negative regulator…
Abstract Number: 1624 • ACR Convergence 2022
Blocking IL-1, IL-33 and IL-36 Signaling with the Anti-IL1RAP Antibody mCAN10 Ameliorates Inflammation and Fibrosis in Preclinical Models of Systemic Sclerosis
Caitriona Grönberg¹, Sara Rattik¹, Meik Kunz², Thoung Trinh-Minh³, Cuong Tran-Manh³, Xiang Zhou³, Petter Skoog¹, David Liberg¹ and Jörg Distler³, ¹Cantargia AB, LUND, Sweden, ²Friedrich-Alexander University (FAU) Erlangen-Nuremberg, Erlangen, Germany, ³Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany
Background/Purpose: The IL-1 receptor accessory protein (IL1RAP) is a co-receptor required for signaling through the IL-1, IL-33, and IL-36 receptors. IL1RAP-dependent signaling has been implicated…
Abstract Number: 1852 • ACR Convergence 2022
Clinical Efficacy and Safety of Guselkumab Maintenance Therapy in Patients with Moderately to Severely Active Crohn’s Disease: Week 48 Analyses from the Phase 2 GALAXI 1 Study
Silvio Danese¹, Remo Panaccione², David T. Rubin³, Bruce E. Sands⁴, Walter Reinisch⁵, Geert D’Haens⁶, Julián Panés⁷, Susana Gonzalez⁸, Kathleen Weisel⁸, Aparna Sahoo⁸, Mary Ellen Frustaci⁸, Zijiang Yang⁸, William J. Sandborn⁹, Anita Afzali¹⁰, Tadakazu Hisamatsu¹¹, Jane M. Andrews¹² and Brian G. Feagan¹³, ¹Gastroenterology and Endoscopy, IRCCS Ospedale San Raffaele and University Vita-Salute San Raffaele, Milano, Italy, ²Inflammatory Bowel Disease Group, University of Calgary, Calgary, AB, Canada, ³University of Chicago Medicine Inflammatory Bowel Disease Center, Chicago, IL, ⁴Dr. Henry D. Janowitz Division of Gastroenterology, Icahn School of Medicine at Mount Sinai, New York, NY, USA, Chicago, IL, ⁵Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria, ⁶Department of Gastroenterology, Amsterdam University Medical Centers, Amsterdam, Netherlands, ⁷Hospital Clínic de Barcelona, IDIBAPS, CIBERehd, Barcelona, Spain, ⁸Immunology, Janssen Research & Development, LLC, Spring House, PA, ⁹Division of Gastroenterology, University of California San Diego, La Jolla, CA, USA, La Jolla, ¹⁰Wexner Medical Center, The Ohio State University, Columbus, OH, ¹¹Department of Gastroenterology and Hepatology, Kyorin University, Tokyo, Japan, ¹²Department of Gastroenterology & Hepatology, Royal Adelaide Hospital & University of Adelaide, Adelaide, Australia, ¹³Western University and Senior Scientific Director, Alimentiv Inc (formerly Robarts Clinical Trials Inc), London, ON, Canada
Background/Purpose: GALAXI 1 is a Phase 2, double-blind, placebo (PBO)-controlled, multicenter study evaluating the efficacy/safety of guselkumab (GUS), a selective IL-23 p19 antagonist, in patients…
Abstract Number: 2180 • ACR Convergence 2022
Transfer of Monoclonal ACPAs Induces Pain, Bone Loss and Tenosynovitis in Mice in a Peptidyl Arginine Deiminase-4 Dependent Manner
Akilan Krishnamurthy¹, Jitong Sun², Alexandra Circiumaru¹, Yogan Kisten¹, Koji Sakuraba³, Thuy Tran¹, Peter Damberg¹, Patrik Jarvoll¹, Li Lu¹, Katalin Sándor¹, Tunhe Zhou⁴, vivianne malmström¹, Camilla I. Svensson¹, Aase Hensvold¹, Anca Catrina¹, Lars Klareskog¹ and Bence Réthi¹, ¹Karolinska Institutet, Stockholm, Sweden, ²Karolinska Institutet, Solna, Sweden, ³Karolinska Institutet and Karolinska University Hospital, Fukuoka, Japan, ⁴Stockholm University, Stockholm, Sweden
Background/Purpose: The appearance of anti-citrullinated protein antibodies (ACPAs) in the circulation represents a major risk factor for developing rheumatoid arthritis (RA). Here we studied whether…
Abstract Number: 0176 • ACR Convergence 2022
Diagnostic Accuracy and Clinical Relevance of Muscle Biopsy in Patients with Suspected Myositis
Kastriot Kastrati¹, Nasim NAKHOST LOTFI², Marwa Tawfik³, Ellen Gelpi², Romana Hoeftberger², Hans-Peter Kiener², Daniel Aletaha⁴, Michael Bonelli⁵ and Helga Radner⁵, ¹Division of Rheumatology, Vienna, Austria, ²Medical University Vienna, Vienna, Austria, ³Suez Canal University, Ismailia, Egypt, ⁴Medical University Vienna, Wien, Austria, ⁵Division of Rheumatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
Background/Purpose: Diagnosis of idiopathic inflammatory myopathies (IIM) represents a clinical challenge and muscle biopsy is regarded as a fundamental procedure for confirming diagnosis. However, the…

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