Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 0082 • ACR Convergence 2025
IBI3011, a Humanized anti-IL1RAP Monoclonal Antibody, Inhibits IL1, IL33, IL36-driven Inflammation Pathway, and Attenuates Inflammation in Preclinical Inflammatory Disease Model
Hongling Tian, Lidan Liang, Chenjuan Zhu, Bin Li, Lei Cao, Meng Ni, Fan Chen, Zhimin Zhang, Min Wu, Tong Liu, Jinyang Li, Yao Xiong, Li Li, Shuaixiang Zhou, Enhong Zhong and Huizhong Xiong, Innovent Biologics (Suzhou) Co., Ltd., Suzhou, Jiangsu, China (People's Republic)
Background/Purpose: Interleukin 1 receptor accessory protein (IL1RAP) serves as a co-receptor that forms receptor complexes with IL1R1, ST2, and IL36R and mediates signaling pathways triggered…
Abstract Number: 2152 • ACR Convergence 2025
Blau Syndrome: features beyond the classic triad and proposal for clinical criteria
Narendra Bagri¹, Nawazish Shaikh², Manisha jana³, Sushain kalsotra⁴, Krithika Rajkumar², Madhumita Roy Chowdhury², Neerja Gupta², Danveer Bhadu⁵, Vishal Gupta² and Rohan Chawla², ¹All India Institute of Medical Sciences(AIIMS), New Delhi, Delhi, India, ²All India Institute of Medical Sciences, Delhi, Delhi, India, ³ALL INDIA INSTITUTE OF MEDICAL SCIENCES, Delhi, ⁴All India Institute of Medical Sciences, Delhi, India, ⁵All India Institute of Medical Sciences, New Delhi, New Delhi, Delhi, India
Background/Purpose: This study describes the characteristic clinical and radiological findings and outcomes of patients with Blau syndrome from a single tertiary care hospital.Methods: A retrospective…
Abstract Number: 1877 • ACR Convergence 2025
Demographics and trends of Inflammatory Arthritis-Related Mortality in the United States, 1999–2019
Aishwarya Sudheer¹, Ishan Jani², Sarath Lal Mannumbeth Renjithlal² and Qi Wang³, ¹Saint Vincent Hospital, Worcester, MA, ²Rochester General Hospital, Rochester, NY, ³Metrohealth System/Case Western Reserve University, Cleveland, OH
Background/Purpose: The study aims to evaluate the racial, sex-related and urbanization-related trends of deaths in patients with inflammatory polyarthropathies in the United States between 1999…
Abstract Number: 1303 • ACR Convergence 2025
Impact of Fruits and Vegetables Intake on Immune-mediated Inflammatory Diseases：a Two-sample Mendelian Randomization Study
Xuemei Tang, Yuting Zhou, Yuxiao Chen, Xinglin Wu and Qiang Luo, Department of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, China, Chongqing, China (People's Republic)
Background/Purpose: A balanced diet can prevent diseases and promote physical and mental health. Accumulating evidence shows that fruits and vegetables (F&Vs) intake is associated with…
Abstract Number: 0929 • ACR Convergence 2025
SP2H, a Targeted Degrader of STimulator of INterferon Genes (STING), selectively inhibits STING-driven inflammation in vitro and in vivo and improves survival in Trex1-/- mice
Martin Jakobsen¹, Pernille Noer², Kristina Byskov³, Emil Nilsson⁴, Laura Ryø⁵, Claus Olesen² and Richard BETHELL⁶, ¹Aarhus University, Aarhus, Denmark, ²Sulis Therapeutics, Aarhus, Denmark, ³Notify Therapeutics, Aarhus, Denmark, ⁴Sulis Therapeutics, Aarhus C, Denmark, ⁵Aarhus University Hospital, Aarhus, Denmark, ⁶Sulis Therapeutics, Stockholm, Sweden
Background/Purpose: STING plays a critical role at the intersection of innate and adaptive immunity and has been linked to the pathogenesis of SSc and SLE.…
Abstract Number: 0050 • ACR Convergence 2025
TL1A expression is upregulated in rheumatic diseases and anti-TL1A antibody reduces disease symptoms and pathological changes in rat collagen-induced arthritis
Preeyam Patel¹, Matthew Siegel², Emily Lewis³, David Giles³, Justin LaFountaine³, Joshua Friedman³ and Andy Spencer³, ¹Spyre Therapeutics, Waltham, ²Spyre Therapeutics, Menlo Park, CA, ³Spyre Therapeutics, Waltham, MA
Background/Purpose: The development of disease-modifying anti-rheumatic drugs (DMARDs) has reduced disease activity in patients living with rheumatic diseases. Despite this, there is continued unmet need…
Abstract Number: 0400 • ACR Convergence 2024
Osteoclastogenesis from Peripheral Blood Mononuclear Cells in Children with Chronic Nonbacterial Osteomyelitis Are Similar to Those from Healthy Children
Jacqueline Bui¹, Jacob Curry², Jessica Kent³, Payton Danosky⁴, Kellen Sanders⁴, Sriya Paluvayi⁴, Wendy Garcia⁴, Alejandra Ruppe⁴, Megan Cheung⁴, Anna Saack⁴, Xinrui Bao⁴, Audrey Luey⁴, Michelle Kim⁴, Emily McDaniel⁴, Amanda Chiu⁴, Sophia Ahn⁵, Ji-Won Park⁴, Sudheshna Thirunahari⁴, Cammie Wei⁴, Liau Adriel⁵, Sophia Pham⁴, Sadie Van Den Bogaerde⁴, Joshua Scheck⁴, Ian Muse⁶, Ava Klein⁵, Xing Wang⁷, James Cassat⁸ and Yongdong Zhao⁶, ¹Seattle Children’s Research Institute, University of Washington, Bellevue, WA, ²Seattle Children’s Research Institute, Vanderbilt University Medical Center, Seattle, WA, ³Seattle Children’s Research Institute, Medical College of Wisconsin, Seattle, WA, ⁴Seattle Children’s Research Institute, University of Washington, Seattle, WA, ⁵Seattle Children’s Research Institute, University of Washington, Seattle, ⁶Seattle Children's Research institute, Seattle, WA, ⁷Biostatistics Epidemiology and Analytics in Research, Seattle Children’s Research Institute, Seattle, WA, ⁸Vanderbilt University Medical Center, Nashville, TN
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disease in which osteoclastogenesis may play a critical role. Cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) was shown…
Abstract Number: 0999 • ACR Convergence 2024
Clinical and Biological Features of VEXAS Syndrome in Women: Study of 9 French Cases Compared with 263 Men
Rim Bourguiba¹, Valentin Lacombe², Vincent Jachiet³, Thibault Comont⁴, Joris Galland⁵, Mael Heiblig⁶, Alexandre Nguyen⁷, Achille Aouba⁸, Xavier Boulu⁹, Alexandre Curie¹⁰, Pierre Sujobert¹¹, Pierre Hirsch¹², Olivier Kosmider¹³, Arsene Mekinian¹⁴ and Sophie Georgin-lavialle¹⁵, and French VEXAS study group, ¹Hopital des Forces de sécurité de l'interieur, La Marsa, Tunisia, ²Angers University Hospital, Angers, France, ³Service de Médecine intene, Hopital Saint-Antoine, Paris France, Paris, France, ⁴4 Service de médecine interne, Hopital Toulouse, France, Toulouse, France, ⁵Service de médecine interne, Centre hospitalier de Bourg- En-Bresse, France, Bourg en Bresse, France, ⁶Service de médecine interne, Centre Hospitalier Lyon Sud, Lyon, France, ⁷Service de médecine interne, CHU Cean, France, Cean, France, ⁸Service d'immunologie clinique-médecine interne, CHU de Caen Normandie, Caen, France, ⁹8 Service de médecine interne, CHU Amiens Picardie, France, Amiens, France, ¹⁰Service de médecine interne, CH Eure Seine, France, Eure seine, France, ¹¹Service d'hématologie, Centre Hospitalier Lyon Sud, France, Lyon, Tunisia, ¹²Hopital Saint-Antoine, Paris, France, Paris, France, ¹³Hematology Laboratory, Assistance Publique-Hôpitaux de Paris, Centre-Université de Paris Cité, Cochin Hospital, Paris, France, Paris, France, ¹⁴Saint Antoine University Hospital, Paris, France, ¹⁵Sorbonne Université, Department of internal medicine, Tenon Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
Background/Purpose: VEXAS syndrome is an autoinflammatory disease associated with somatic mutations in the UBA1 gene, which is located on the X chromosome, which explains the…
Abstract Number: 1773 • ACR Convergence 2024
Divergent Genetic Architecture in Boys and Girls with NEMO-deleted Exon 5 Autoinflammatory Syndrome (NEMO-NDAS) Implies Role for Wildtype Effector Cells
Adriana Almeida de Jesus¹, Kip Friend², Bin Lin³, Eric Karlins⁴, Colton McNinch⁴, Sara Alehashemi⁵, Keith Kauffman⁶, FARZANA BHUYAN³, Taylor Newbolt⁶, Andrea Bohrer⁷, Andre Rastegar³, Sophia Park³, Dana Kahle³, Jacob Mitchell³, Amanda Chen³, Sofia Torreggiani⁸, Kader Cetin Gedik⁹, Katsiaryna Uss², Amer Khojah¹⁰, Eveline Wu¹¹, Christiaan Scott¹², Timothy Ronan Leahy¹³, Emma MacDermott¹⁴, Orla Killeen¹⁵, Thaschawee Arkachaisri¹⁶, Brian Nolan¹⁷, Zoran Gucev¹⁸, Kathryn Cook¹⁹, Vafa Mammadova²⁰, Gulnara Nasrullayeva²⁰, Mariana Correia Marques²¹, Abigail Bosk²², Seza Ozen²³, Scott Canna²⁴, Maude Tusseau²⁵, Emilie Chopin²⁶, Guilaine Boursier²⁷, Veronique Hentgen²⁸, Ines Elhani²⁹, Mario Sestan³⁰, Marija Jelusic³¹, Danielle Fink³², Douglas Kuhns³², Clifton Dalgard³³, Alexandre Belot³⁴, Timothy Moran¹¹, Katherine Meyer-Barber⁷, Andrew Oler⁴, Daniel Barber⁶ and Raphaela Goldbach-Mansky³⁵, ¹NIAID, NIH, Silver Spring, MD, ²Translational Autoinflammatory Diseases section (TADS), LCIM, NIAID, NIH, Bethesda, MD, ³TADS, NIAID, NIH, Bethesda, MD, ⁴BCBB, NIAID, NIH, Bethesda, MD, ⁵NIH/NIAID/TADS, Potomac, MD, ⁶T Lymphocyte Biology Section, LPD, NIAID, NIH, Bethesda, MD, ⁷Inflammation and Innate Immunity Unit, LCIM, NIAID, NIH, Bethesda, MD, ⁸University Of Maryland Baltimore, Baltimore, MD, ⁹Translational Autoinflammatory Diseases section (TADS), LCIM, NIAID, NIH, Pittsburgh, PA, ¹⁰Umm Al-Qura University, Makkah, Saudi Arabia, ¹¹University of North Carolina School of Medicine, Chapel Hill, NC, ¹²University of Cape Town, Cape Town, South Africa, ¹³Children’s Health Ireland (CHI) at Crumlin, Dublin, Ireland, ¹⁴CHI Crumlin, Dublin, Dublin, Ireland, ¹⁵Children's Health Ireland, Dublin, Ireland, ¹⁶KK Women's and Children's Hospital, SingHealth, Singapore, Singapore, ¹⁷Ann and Robert H Lurie Children's Hospital of Chicago, Chicago, IL, ¹⁸University Children's Hospital, Skopje, Macedonia, ¹⁹Akron Children's Hospital, Akron, OH, ²⁰Azerbaijan Medical University, Baku, Azerbaijan, ²¹National Institutes of Health, Bethesda, MD, ²²Children's National Hospital, Bethesda, DC, ²³Department of Pediatrics, Hacettepe University, Ankara, Turkey, ²⁴Children's Hospital of Philadelphia, Philadelphia, PA, ²⁵RAISE, Hospices Civils de Lyon, Lyon, France, ²⁶Hospices Civils de Lyon, Lyon, France, ²⁷University of Montpellier, Montpellier, ²⁸Laboratoire de Génétique des Maladies Rares et Autoinflammatoires, Département de Génétique Médicale, Maladies Rares et Médecine Personnalisée, CEREMAIA, CHU de Montpellier, Univ Montpellier, Montpellier, France, Le Chesnay, France, ²⁹Department of Internal Medicine, Caen University Hospital, Caen, France, Caen, France, ³⁰University of Zagreb School of Medicine University Hospital Centre Zagreb, Zagreb, Croatia, ³¹University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, ³²Collaborative Clinical Research Branch, NIAID, NIH, Bethesda, MD, Bethesda, MD, ³³The American Genome Center, Department of Anatomy, Physiology & Genetics, Uniformed Services University, Bethesda, MD, ³⁴Hospices Civils de Lyon, Collonges au mont d'or, France, ³⁵Translational Autoinflammatory Diseases section (TADS), LCIM, NIAID, NIH, Potomac, MD
Background/Purpose: Splice-site variants in X-linked IKBKG cause NEMO-deleted exon5 autoinflammatory syndrome (NEMO-NDAS); a pseudogene (IKBKGP1) complicates genetic diagnosis. NEMO-NDAS is four times more common in…
Abstract Number: 2044 • ACR Convergence 2024
Clinical Practice Patterns, Rilonacept Appropriate Use for Recurrent Pericarditis
Jessica Farrell¹, Michael Allen², Lee Shapiro³, Victoria Michaels⁴, Arifa Javed⁵, Nabiha Qureshi⁶, Summia Matin Afridi⁷, Shahad Almahmoud⁸, Getzabeth Bosques Gomez⁹ and Khoa Richard Ngo¹⁰, ¹Albany College of Pharmacy & Health Sciences/Albany Medical Center, Delmar, NY, ²Albany Medical Center, Delmar, NY, ³Albany Medical College, Stillwater, NY, ⁴Albany Medical Center, Wynantskill, NY, ⁵Albany Medical Center, Rensselaer, NY, ⁶Albany Medical Center, Menands, NY, ⁷Albany Medical Center, Albany, NY, ⁸Albany College of Pharmacy & Health Sciences/Albany Medical Center, Albany, NY, ⁹Albany College of Pharmacy and Health Sciences/Albany Medical Center, Albany, NY, ¹⁰Albany Medical Center, Voorheesville, NY
Background/Purpose: Pericarditis is inflammation of the pericardial sac which can be of infectious or non-infectious etiology, commonly associated with rheumatic diseases. Pericarditis is defined as…
Abstract Number: 2659 • ACR Convergence 2024
A Comprehensive Approach Utilizing a Combination of Genetics, Cytological Analysis, and Imaging Techniques to Speed up the Diagnostic Process in VEXAS Patients
Sara Bindoli¹, Chiara Baggio², Roberto Padoan³, Riccardo Bixio⁴, doria Andrea⁵, Roberta Ramonda² and Paolo Sfriso¹, ¹Rheumatology Unit, University of Padova, Italy, Padova, Veneto, Italy, ²Rheumatology Unit, University of Padova, Italy, Padova, Italy, ³Department of Medicine DIMED, Division of Rheumatology, University of Padua, Padua, Italy, ⁴University of Verona, Verona, Verona, Italy, ⁵University of Padova, Padova, Italy
Background/Purpose: VEXAS is a newly described “haemato-autoinflammatory” condition resulting from somatic mutations in the UBA-1 gene. By integrating genetics, cytology and imaging, this work seeks…
Abstract Number: 0006 • ACR Convergence 2024
NX-5948, a Clinical-Stage BTK Degrader, Achieves Deep Suppression of BCR, TLR, and FcR Signaling in Immune Cells and Demonstrates Efficacy in Preclinical Models of Arthritis and Other Inflammatory Diseases
Mark Noviski¹, Jun Ma¹, Nivetha Brathaban¹, Aishwarya Kumar¹, Dhwani Haria¹, Jenny McKinnell¹, Robert Cass¹, Frederick Cohen¹, Davorka Messmer², Gwenn Hansen¹ and Ryan Rountree¹, ¹Nurix Therapeutics, San Francisco, CA, ²Nurix Therapeutics, San Diego, CA
Background/Purpose: Bruton’s tyrosine kinase (BTK) mediates signaling downstream of the B cell receptor (BCR), toll-like receptors (TLRs), and Fc receptors (FcRs). This makes BTK an…
Abstract Number: 0408 • ACR Convergence 2024
Analysis of Protein Biomarkers for the Prediction of IL-1 Inhibitor Treatment Response in the CARRA First-line Options for Systemic Juvenile Idiopathic Arthritis Treatment (FROST) Study
Michael Matt¹, Mariana Correia Marques², Sabrina Fuehner³, Lexi Auld¹, George Tomlinson⁴, Michael Ombrello⁵, Yukiko Kimura⁶, Christoph Kessel⁷ and grant schulert¹, and the CARRA FROST Investigators, ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²National Institutes of Health, Bethesda, MD, ³Universitaetsklinikum Muenster, Muenster, Germany, ⁴University of Toronto, Toronto, ON, Canada, ⁵National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ⁶Hackensack Meridian School of Medicine, New York, NY, ⁷University Hospital Muenster, Muenster, Germany
Background/Purpose: IL-1 inhibitors are both widely used and highly effective as first-line therapy for children with systemic juvenile idiopathic arthritis (sJIA); however, the mechanisms underlying…
Abstract Number: 1062 • ACR Convergence 2024
Assessing Musculoskeletal Symptom Burden in a Cohort of Patients with Hidradenitis Suppurativa Using the IDEOM MSK-Q
Gretchen Ball¹, Hassan Hamade², Sarah Romanelli², Zachary Levy², Lourdes Perez Chada³, Joseph Merola⁴ and Alice Gottlieb⁵, ¹Albert Einstein College of Medicine, Ridgefield, CT, ²Icahn School of Medicine at Mount Sinai, New York, NY, ³Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, ⁴UT Southwestern Medical Center, Newton, MA, ⁵Icahn School of Medicine at Mount Sinai, Department of Dermatology, Seattle, WA
Background/Purpose: Hidradenitis suppurativa (HS) has been associated with inflammatory joint diseases such as psoriatic arthritis and ankylosing spondylitis. Despite this correlation, dermatologists do not routinely…
Abstract Number: 1778 • ACR Convergence 2024
A Novel Variant in IRAK2 Results in Immune Dysregulation in Systemic Juvenile Idiopathic Arthritis (sJIA)
Mariana Correia Marques¹, Brooke Boyd², Alana Platukus³, Elizabeth Schmitz², Hiroto Nakano², Faiza Naz⁴, Anthony Cruz¹, Stefania Dell'Orso⁴, Zuoming deng⁴ and Michael Ombrello⁵, ¹National Institutes of Health, Bethesda, MD, ²Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, MD, ³1Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, MD, ⁴National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, Bethesda, MD, ⁵National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD
Background/Purpose: Lung disease (LD) is poorly understood complication of Still’s disease with high fatality. HLA-DRB1*15 is a strong risk factor for the development of this…

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