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: 2151 • ACR Convergence 2025
Real-World Experience with IL-1 Blockade in Children with Undifferentiated Systemic Autoinflammatory Diseases (uSAIDs)
Maryam Ashoor¹, Kosar Asna Ashari², Kyle McBrearty², Joyce Chang³, Jonathan Hausmann⁴ and Fatma Dedeoglu², ¹Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Brookline, MA, ²Boston Children's Hospital, Boston, MA, ³Boston Children's Hospital, Boston, ⁴Boston Children's Hospital, bosto, MA
Background/Purpose: Systemic Autoinflammatory Diseases (SAIDs) are a group of rare disorders caused by dysregulation of the innate immune system. Over 50 genes have been identified…
Abstract Number: 1827 • ACR Convergence 2025
Transcriptomics and Machine Learning Unraveling the Molecular Drivers of PFAPA Flares
Sivia Lapidus¹, Tresa Ambooken², Ellen Hakim³, Tara Lozy⁴, Elahe Golalipour³, Sohail Adonimohammed⁵, Jennifer Weiss⁶, Suzanne Li⁶, Amanda Nowakowski⁷, Aryeh Lejtman⁸, Aaron Sebbag³, Ariel Aptekmann⁹ and Jigar Desai⁹, ¹Joseph M. Sanzari Children's Hospital, Center for Discovery and Innovation, Hackensack Meridian School of Medicine, Montclair, NJ, ²Food and Drug Administration, Floral Park, NY, ³Center for Discovery and Innovation, Hackensack Meridian Health, Nutley, NJ, ⁴Joseph M. Sanzari Children's Hospital, Center for Discovery and Innovation, Hackensack Meridian School of Medicine, Nutley, ⁵Hackensack Meridian School of Medicine, Nutley, NJ, ⁶Joseph M. Sanzari Children's Hospital and Hackensack Meridian School of Medicine, Hackensack, NJ, ⁷Joseph M. Sanzari Children's Hospital, Hackensack Meridian Health, Hackensack, NJ, ⁸Joseph M. Sanzari Children's Hospital and Center for Discovery and Innovation, Hackensack Meridian Health, Nutley, NJ, ⁹Center for Discovery and Innovation and Hackensack Meridian School of Medicine, Hackensack Meridian Health, Nutley, NJ
Background/Purpose: Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) is the most common periodic fever presenting with frequent episodes of pain and impaired function. While…
Abstract Number: 1302 • ACR Convergence 2025
Dose Adjustment was Necessary in Patients with Cryopyrin-Associated Periodic Syndrome Switching from Anakinra to Canakinumab
Soyoung Lee and Hyun Kyung Lee, Chung-Ang University Hospital, Seoul, Republic of Korea
Background/Purpose: Cryopyrin-associated periodic syndrome (CAPS) is a spectrum of rare autoinflammatory disorders caused by mutations in the NLRP3 gene, leading to excessive interleukin-1β (IL-1 β)…
Abstract Number: 0920 • ACR Convergence 2025
Functional Characterization of NEMO-NDAS Causing Variants in Patients’ PBMCs and in Wildtype and Mutant U937 Cells
Elizabeth Morgan¹, Bin Lin², Sara alehashemi¹, Adriana de Jesus¹, Keith Kauffman³, Christopher Friend¹, Farzana Bhuyan¹, Kader Gedik¹, Kat Uss¹, Lauren Krausfeldt⁴, Jason Brenchley⁵, Zoran Gucev⁶, Kathryn Cook⁷, Vafa Mammadova⁸, Gulnara Nasrullayeva⁸, Mariana Correia Marques⁹, Abigail Bosk¹⁰, Brian Nolan¹¹, Scott Canna¹², Maude Tusseau¹³, Andrea Bohrer¹⁴, Katrin Mayer-Barber¹⁵, Timothy Moran¹⁶, Andrew Oler⁴, Daniel Barber³ and Raphaela Goldbach-Mansky¹, ¹Translational Autoinflammatory Diseases Section (TADS), Laboratory of Clinical Immunology and Microbiology (LCIM), NIAID, NIH, Bethesda, MD, ²NIH, Bethesda, MD, ³T-Lymphocyte Biology Section, Laboratory of Parasitic Diseases, NIAID, NIH, Bethesda, MD, ⁴Bioinformatics and Computational Biosciences Branch, Office of Cyber Infrastructure and Computational Biology, NIAID, NIH, Bethesda, MD, ⁵Barrier Immunity Section, Laboratory of Viral Diseases, NIAID, NIH, Bethesda, MD, ⁶University Children's Hospital, Medical Faculty Skopje, Skopje, Macedonia, ⁷Akron Children’s Hospital, Akron, OH, ⁸Azerbaijan Medical University, Baku, Azerbaijan, ⁹Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ¹⁰Children’s National Hospital, Washington DC, ¹¹Lurie Children’s Hospital, Chicago, ¹²Children's Hospital of Philadelphia, Philadelphia, PA, ¹³Hôpital Femme-Mère-Enfant, Bron, France, ¹⁴Inflammation and Innate Immunity Unit, Laboratory of Clinical Immunology and Microbiology (LCIM), NIAID, NIH, Bethesda, MD, ¹⁵Inflammation and Innate Immunity Unit, Laboratory of Clinical Immunology and Microbiology (LCIM), Bethesda, MD, ¹⁶University of North Carolina School of Medicine, Chapel Hill, NC
Background/Purpose: NEMO-deleted 5 autoinflammatory syndrome (NEMO-NDAS) is an inflammatory disease caused by mosaic splice-site variants that lead to exon 5 skipping in IKBKG, encoding NEMO.…
Abstract Number: 0030 • ACR Convergence 2025
Immune-related Diagnoses Associated with NOD2 Variants in Human Subjects: A Phenome-wide Association Study
John Davis¹, Elizabeth Atkinson¹, Vanessa Kronzer¹, Cynthia Crowson², Afsaneh Alavi³, John Damianos¹, Loftus Edward¹, Joseph Murray¹, Ann Moyer¹ and Filippo Pinto e Vairo¹, ¹Mayo Clinic, Rochester, MN, ²Mayo Clinic, Stewartvillle, MN, ³Department of Dermatology, Mayo Clinic, Rochester, MN
Background/Purpose: The nucleotide-binding oligomerization domain-containing protein 2 (NOD2) gene is associated with risk for several inflammatory diseases, including Crohn disease, Blau syndrome, and Yao syndrome…
Abstract Number: 2141 • ACR Convergence 2025
Treatment of Refractory Still’s/Systemic Juvenile Idiopathic Arthritis Lung Disease with the bi-specific IL-1Beta/IL-18 neutralizing antibody MAS825
Hallie Carol¹, Monica Manglani², Abass Noor², Edward Behrens³, Jon Burnham⁴, Jessica Lee², Jordan Moreno², Alexandra Chop⁵, Kader Cetin Gedik⁶, Catherine Poholek⁷, Ginger Janow⁸, Lindsay Waqar-Cowles¹ and Scott Canna¹, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²Children's Hospital of Philadelphia, Philadelphia, ³CHOP, West Chester, PA, ⁴Children's Hospital of Philadelphia, Bryn Mawr, PA, ⁵UPMC, Pittsburgh, PA, ⁶UPMC Children's Hospital of Pittsburgh/University of Pittsburgh, Pittsburgh, PA, ⁷University of Pittsburgh, Pittsburgh, PA, ⁸Joseph M. Sanzari Children's Hospital at Hackensack Meridian Health, Hackensack, NJ
Background/Purpose: Despite major advances in the understanding, diagnosis, and treatment of Still’s disease (including Systemic Juvenile Idiopathic Arthritis, sJIA), many patients experience a refractory course.…
Abstract Number: 1824 • ACR Convergence 2025
Tissue-Resident Natural Killer Cells May Drive Monocyte Differentiation And Macrophage Accumulation In The Inflamed Joints Of Pediatric Juvenile Idiopathic Arthritis Patients
Roselyn Fierkens¹, Jun Inamo², Clara Lin³, Nathan Rogers⁴, Kari Hayes¹, Heather Leach³ and Kentaro Yomogida¹, ¹University of Colorado, Aurora, CO, ²University of Colorado School of Medicine, Aurora, CO, ³Children's Hospital Colorado, Aurora, ⁴Children’s Hospital Colorado, Aurora, CO
Background/Purpose: Natural Killer (NK) cells are the most abundant innate lymphoid cells, accounting for 10–40% of total lymphocytes in peripheral blood. They adapt to diverse…
Abstract Number: 1191 • ACR Convergence 2025
Effect of ER Stress Inhibition With 4-Phenylbutyric Acid on Disease Phenotype in a Mouse Model of Myositis
Alfredo Guzman, Elizabeth Bagley, Rita Spathis, Madison King, Kanneboyina Nagaraju and Melissa Morales, Binghamton University, Johnson City, NY
Background/Purpose: Dysregulation of the ER stress and interferon (IFN) pathways play a major role in the pathophysiology of autoimmune myositis. Upregulation of ER stress markers…
Abstract Number: 0918 • ACR Convergence 2025
Retention of variant forms of TNFR1 in the Golgi induces stress responses and monocyte activation in systemic Juvenile Idiopathic Arthritis (sJIA)
Anthony Cruz¹, Krisztian Csomos¹, Boglarka Ujhazi¹, Hiroto Nakano², Marissa Krantz³, Sophia Chou⁴, Emily Rosenbaum¹, Tianmin Fu⁵, Dirk Foell⁶, Rae Yeung⁷, Pamela Weiss⁸, Faiza Naz⁹, Ly-Lan Bergeron¹, Michelle Millwood¹⁰, Carol Lake¹¹, Emely Verweyen⁶, Grant Schulert¹², Stefania Dell'orso⁹, Hao Wu¹³, Zuoming Deng¹⁴, davide Randazzo¹⁵ and Michael Ombrello¹⁶, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Bethesda, MD, ²NIAMS, NIH, Bethesda, MD, ³University of Rochester, Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Rockville, MD, ⁵Ohio State University College of Medicine, Columbus, OH, ⁶University Hospital Muenster, Muenster, Germany, ⁷The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, ⁸Childrens Hospital of Philadelphia, Philadelphia, PA, ⁹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ¹⁰National Institutes of Health (NIH), Bethesda, MD, ¹¹NIH, GAITHERSBURG, MD, ¹²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ¹³Harvard University, Cambridge, MA, ¹⁴Biodata Mining and Discovery Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ¹⁵Light Imaging Section, Office of Science and Technology, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, ¹⁶National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is severe and poorly understood inflammatory condition. Tumor necrosis factor receptor 1 (TNFR1) is activated by TNF and is…

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