Abstracts tagged "innate immunity"

Abstract Number: 1824 • ACR Convergence 2024
Skin Macrophage Subtypes and Impact of Tofacitinib in Early Diffuse Cutaneous Systemic Sclerosis: Results from Single-cell Analyses of an Observational Data Set and a Phase I/II Randomized Controlled Trial
Juliette Ferrant¹, Alain Lescoat², Valérie Lecureur³, Marie Lelong³, John Varga⁴, Robert Lafyatis⁵, Johann Gudjonsson⁴ and Dinesh Khanna⁴, ¹CHU Rennes, Rennes, France, ²CHU Rennes - University Rennes 1, Rennes, France, ³Rennes University, Rennes, France, ⁴University of Michigan, Ann Arbor, MI, ⁵University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Macrophages play a major role in dcSSc-related skin fibrosis, with a mixed M1-M2 activation profile relying on the activation of JAK/STAT. Tofacitinib, a pan-JAK…
Abstract Number: 0072 • ACR Convergence 2024
The Association Between Activation of Inflammasome and HLA-B27 in Dendritic Cells of Patients with Axial Spondyloarthritis
Jina Yeo¹, Min-Gang Kim², Hye Eun Lee³, Yeonsoo Yang², Mi Ryoung Seo¹, Hyo-Jin Choi¹, Eun Young Lee⁴, Dae Ho Lee³ and Han Joo Baek⁵, ¹Division of Rheumatology, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, Republic of Korea, ²Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Republic of Korea, ³Division of Endocrinology, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, Republic of Korea, ⁴3Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea, ⁵Division of Rheumatology, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Inchon, South Africa
Background/Purpose: Axial spondyloarthritis (axSpA) exhibits a predominant involvement of the innate immune system, marked by abnormal functions of innate immune cell such as dendritic cells…
Abstract Number: 1863 • ACR Convergence 2024
Human Endogenous Retroviruses Promote the Aberrant T Cell Differentiation in Systemic Lupus Erythematosus
Xiaoli Min¹, Jiali Wu², Yaqin Yu³, Qianjin Lu⁴ and Ming Zhao⁴, ¹Institute of Dermatology, Chinese Academy of Medical Sciences, Nanjing, China (People's Republic), ²Hunan Key Laboratory of Medical Epigenomics, Second Xiangya Hospital, Central South University, Changsha, China (People's Republic), ³Central South University, Changsha, China (People's Republic), ⁴Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, China (People's Republic)
Background/Purpose: Background Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple organs and systems. The complex pathogenesis of SLE involved the abnormal activation of CD4+T cells and DNA hypomethylation modification. Human endogenous retroviruses (HERVs)…
Abstract Number: 0305 • ACR Convergence 2024
Inflammatory T Cell Expansion in Yao Syndrome
Danielle Xie¹, Rimanpreet Kaur², Richard Kew², Qingping Yao³ and Charles Vorkas², ¹Stony Brook University, East Setauket, ²Stony Brook University, Stony Brook, ³Stony Brook University, Stony Brook, NY
Background/Purpose: Yao syndrome (YAOS, #OMIM 617321), formerly known as nucleotide-binding oligomerization protein containing 2 (NOD2)-associated autoinflammatory disease, is associated with specific NOD2 mutations and affects…
Abstract Number: 2199 • ACR Convergence 2024
Mycobacterial Infection and Renal and Bladder Malignancy in 2 IFNopathy Patients on High Doses of JAK Inhibitors
Sara Alehashemi¹, Kader Cetin Gedik², Cassandra Calabrese³, Sherry Nguyen⁴, Alexi Baumgardner⁴, Katsiaryna Uss⁴, Kip Friend⁴, Ariane Soldatos⁵, Adriana Almeida de Jesus⁶ and Raphaela Goldbach-Mansky⁷, ¹NIH/NIAID/TADS, Potomac, MD, ²Translational Autoinflammatory Diseases section (TADS), LCIM, NIAID, NIH, Pittsburgh, PA, ³Cleveland Clinic Foundation, Cleveland Heights, OH, ⁴Translational Autoinflammatory Diseases section (TADS), LCIM, NIAID, NIH, Bethesda, MD, ⁵NINDS/NIH, Bethesda, MD, ⁶NIAID, NIH, Silver Spring, MD, ⁷Translational Autoinflammatory Diseases section (TADS), LCIM, NIAID, NIH, Potomac, MD
Background/Purpose: Autoinflammatory Type I Interferonopathies (IFNopathies) include STINGopathies (e.g., SAVI and COPA syndrome), proteasomopathies (e.g., CANDLE/Proteasome associated autoinflammatory syndrome (PRAAS), and oligonucleotidopathies (e.g., AGS: Aicardi-Goutières…
Abstract Number: 0924 • ACR Convergence 2024
The Cytosolic DNA Sensor AIM2 Regulates Bone Homeostasis Through Bone Progenitor Cell Differentiation
Jia (Sijia) Chen¹, Yukiko Maeda², Catherine Manning³, Victor Le⁴, Jae-Hyuck Shim⁵, Katherine A. Fitzgerald⁶ and Ellen Gravallese⁷, ¹Brigham and Women's Hospital, Boston, MA, ²Sanofi, worcester, MA, ³Brigham and Women's Hospital, Dedham, MA, ⁴Digital Biology, Boston, MA, ⁵University of Massachusetts, Worcester, MA, ⁶UMass Chan Medical School, Worchester, MA, ⁷Brigham and Women's Hospital, Harvard Medical School, Chestnut Hill, MA
Background/Purpose: Innate sensing pathways and inflammasomes play an important role in autoimmunity and aging. AIM2 is a DNA sensor that triggers inflammasome assembly in myeloid…
Abstract Number: 2327 • ACR Convergence 2024
Differential Quantitative Profile of Myeloid Derived Suppressor Cells and Monocyte Subsets Relate to Clinical Phenotype and Disease Activity in Psoriatic Arthritis Patients
Jennifer Balderas Miranda¹, Jiram Torres Ruiz², Guillermo Guaracha Basañez³, Virginia Pascual Ramos⁴, Beatriz Alcalá-Carmona⁵, Yatzil Reyna-Juárez⁵, María José Ostos-prado⁵, Nancy R Mejía-Domínguez⁵, Guillermo Juarez-Vega⁶ and Diana Gomez-martin⁷, ¹Universidad Nacional Autónoma de México, Ciudad de México, Mexico, ²INCMNSZ, Mexico, Federal District, Mexico, ³Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirón", Mexico City, Mexico, ⁴Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Federal District, Mexico, ⁵Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Distrito Federal, Mexico, ⁶Red de Apoyo a la Investigación, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán and Universidad Nacional Autónoma de México, Mexico City, Distrito Federal, Mexico, ⁷INCMNSZ, Mexico City, Federal District, Mexico
Background/Purpose: The innate immune system not only participates in PsA pathogenesis but may also counteract with immunomodulatory elements. Proinflammatory and repairing roles according to monocyte…
Abstract Number: 0932 • ACR Convergence 2024
IRAK4 Degrader GS-6791 Inhibits TLR and IL-1R-Driven Inflammatory Signaling, and Ameliorates Disease in a Preclinical Arthritis Model
Grace Teng¹, Thomas Fung², Annamaria Mocciaro², Ceyda Llapashtica¹, Angie Hammond³, Jesse Gurgel⁴, zhiyu Huang¹, Maria Mouchess¹, Vanessa Gorney⁵, Wesley Minto¹, Sunish Mohanan¹, Adam Schrier¹, Wylie Palmer², Alexandra Borodovsky² and Gundula Min-oo¹, ¹Gilead Sciences, Foster City, CA, ²Nurix Therapeutics, San Francisco, CA, ³Gilead Sciences, Inc., Seattle, WA, ⁴Gilead Sciences, Seattle, CA, ⁵Alterome Therapeutics, San Diego, CA
Background/Purpose: Despite advances in treatment, chronic inflammatory diseases such as rheumatoid arthritis (RA) represent areas of high unmet medical need. IRAK4 is a proximal mediator…
Abstract Number: 2505 • ACR Convergence 2024
C5 Signaling Pathway Genes as Key Drivers of the Pathogenesis of IgA Vasculitis?
Joao Carlos Batista-Liz¹, Vanesa Calvo-Rio², María Sebastián Mora-Gil³, Ligia Gabrie-Rodriguez⁴, Rafael Gálvez Sánchez⁴, Belén Sevilla-Pérez⁵, José Luis Callejas⁶, María Teresa Leonardo⁷, Ana Peñalba⁷, Javier Narvaez-García⁸, Luis Martín-Penagos⁹, Luis Caminal-Montero¹⁰, PAZ COLLADO¹¹, Patricia Quiroga Colina¹², Esther Vicente-rabaneda¹³, Esteban Rubio¹⁴, Manuel León Luque¹⁵, Juan María Blanco-Madrigal¹⁶, Eva Galindez-Agirregoikoa¹⁷, Santos Castañeda¹³, Ricardo Blanco-Alonso¹⁸, Veronica Pulito-Cueto¹ and Raquel Lopez-mejias¹, ¹IDIVAL, Santander, Spain, ²Valdecilla Hospital, Santander, Cantabria, Spain, ³Immunopathology Group, IDIVAL, Santander, Spain, ⁴Immunopathology Group, IDIVAL. Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁵Division of Pediatrics, Hospital Universitario San Cecilio, Granada, Spain, ⁶Systemic Autoimmune Diseases Unit, Hospital Clinico San Cecilio, Instituto de Investigación Biosanitaria de Granada ibs.GRANADA, Granada, Spain, ⁷Division of Pediatrics, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁸Hospital Universitario de Bellvitge, Barcelona, Spain, ⁹Immunopathology Group, IDIVAL. Division of Nephrology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ¹⁰Internal Medicine Department, Hospital Universitario Central de Asturias, Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Oviedo, Spain, ¹¹Hospital Universitario Severo Ochoa, MADRID, Spain, ¹²Division of Rheumatology, Hospital Universitario de La Princesa, Madrid, Spain, ¹³Hospital Universitario de La Princesa, Madrid, Spain, ¹⁴Division of Rheumatology, Hospital Universitario Virgen del Rocío, Sevilla, Spain, Sevilla, Spain, ¹⁵Division of Rheumatology, Hospital Universitario Virgen del Rocío, Sevilla, Spain., Sevilla, Spain, ¹⁶Division of Rheumatology, Hospital Universitario de Basurto, Bilbao, Spain., Bilbao, Spain, ¹⁷BASURTO UNIVERSITY HOSPITAL, BILBAO, Spain, ¹⁸Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain
Background/Purpose: Immunoglobulin A Vasculitis (IgAV) is an inflammatory disease caused by the accumulation of immune complexes of IgA in the walls of small blood vessels…
Abstract Number: L02 • ACR Convergence 2023
Mutational Analysis of UNC93B1 Identifies Regulatory Regions Mutated in Human SLE
John Huizar¹, Victoria Rael², Julian Yano², Leianna Slayden³, Madeleine Weiss⁴, Robert Saxton⁴, Bo Liu⁵, Olivia Majer⁶ and Gregory Barton⁷, ¹UCSF, San Francisco, CA, ²Division of Immunology and Molecular Medicine, Department of Molecular and Cell Biology, UC Berkeley, Berkeley, CA, ³HHMI at UC Berkeley, Berkeley, CA, ⁴UC Berkeley, Berkeley, CA, ⁵Institut Pasteur of Shanghai, Chinese Academy of Sciences, Shangai, China, ⁶Max Planck Institute for Infection Biology, Berlin, Germany, ⁷UC Berkeley / HHMI, Berkeley, CA
Background/Purpose: Endosomal nucleic acid-sensing toll-like receptors 3, 7 and 9 are key innate immune sensors of dsRNA, ssRNA and ssDNA, respectively, whose activities must be…
Abstract Number: L08 • ACR Convergence 2023
Early Clinical Development of CIT-013, a First in Class NETosis Inhibitor, in a Randomized Phase I Dose Escalation Study in Healthy Volunteers Demonstrating Potent Inhibition of LPS Induced Neutrophil Extracellular Trap Formation
Leonie Middelink¹, Renato Chirivi², Helmuth van Es², Eric Meldrum², Peter van Zandvoort², Tirza Bruurmijn², Matthijs Moerland³ and Patrick Round², ¹Middelinc., Utrecht, Netherlands, ²Citryll BV, Oss, Netherlands, ³CHDR, Leiden, Netherlands
Background/Purpose: Aberrant Neutrophil Extracellular Traps (NETs) production contributes to the pathophysiology of multiple inflammatory and autoimmune diseases such as Rheumatoid arthritis (RA) Hidradenitis suppurativa (HS),…
Abstract Number: 0757 • ACR Convergence 2023
Identification of 23 Novel COPA Rare Exonic Non-Synonymous Variants and Their Associated Autoimmune and Inflammatory Clinical Phenotypes Among 53,364 Individuals
Pierre-Antoine Juge¹, Gregory McDermott², Keigo Hayashi², Jing Cui³, Clémence David-Gabarre⁴, Marie-Louis Frémond⁴, Holly Wobma⁵, Soumya Raychaudhuri², Elizabeth Karlson², Philippe Dieudé⁶ and Jeffrey Sparks⁷, ¹Division of Rheumatology, Inflammation, and Immunity Brigham & Women's Hospital, Boston, MA, ²Brigham and Women's Hospital, Boston, MA, ³Brigham and Women's Hospital, Boston, MA, ⁴Imagine, Paris, France, ⁵Division of Immunology, Boston Children's Hospital, Boston, MA, ⁶Assistance Publique-Hôpitaux de Paris, Bichat-Claude Bernard University Hospital, INSERM UMR1152, University de Paris Cité, Department of Rheumatology, Paris, France, ⁷Division of Rheumatology, Inflammation, and Immunity, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA
Background/Purpose: COPA syndrome is a rare, severe monogenic autoinflammatory/autoimmune disorder with variable penetrance, most commonly affecting the lungs and joints. It is caused by dominant…
Abstract Number: 1712 • ACR Convergence 2023
Identification and Functional Characterization of Eight CANDLE/PRAAS Causing Proteasome Variants in Five Unrelated Patients
Adriana Almeida de Jesus¹, Jonas J. Papendorf², Frederic Ebstein³, Sara Alehashemi⁴, Daniela Pioto⁵, Anna Kozlova⁶, Maria Teresa TErreri⁷, Anna Shcherbina⁶, Andre Rastegar⁸, Marta Rodrigues⁹, Renan Pereira¹⁰, Sophia Park¹¹, Bin Lin¹², Kat Uss¹¹, Ana Flavia da Silva Pina⁵, FLAVIO SZTAJNBOK¹³, Sofia Torreggiani¹⁴, Jennifer Stoddard¹⁵, Julie Niemela¹⁵, Sergio Rosenzweig¹⁵, Adriana Fonseca¹⁶, Marietta De Guzman¹⁷, Nicole Micheloni⁵, Melissa Fraga⁵, Sandro Perazzio¹⁸, Raphaela Goldbach-Mansky¹⁹ and Elke Krueger², ¹NIAID, NIH, Bethesda, MD, ²Institut für Medizinische Biochemie und Molekularbiologie (IMBM), Universitätsmedizin Greifswald, Greifswald, Germany, ³Nantes Université, Nantes, France, ⁴NIH/NIAID/TADS, Clarksville, MD, ⁵Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil, ⁶Center for Pediatric Hematology, Oncology, Immunology, Moscow, Russia, ⁷UNIFESP, São Paulo, Brazil, ⁸NIH, Bethesda, MD, ⁹Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil, ¹⁰Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil, ¹¹Translational Autoinflammatory Diseases Section, LCIM, NIAID, NIH, Bethesda, MD, ¹²Translational Autoinflammatory Diseases Section l LCIM, NIAID, NIH, Bethesda, MD, ¹³UFRJ/UERJ, São Paulo, Brazil, ¹⁴University of Maryland Baltimore, Baltimore, MD, ¹⁵Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, ¹⁶Instituto de Puericultura e Pediatria Martagao Gesteira / Universidade Federal do Rio de Janeiro, Rio De Janeiro, Brazil, ¹⁷Baylor College of Medicine, Houston, TX, ¹⁸Universidade de Sao Paulo (Unifesp); Universidade de São Paulo (USP); Fleury Laboratories, São Paulo, Brazil, ¹⁹NIH/NIAID, Potomac, MD
Background/Purpose: Mutations in genes coding for 20S proteasome subunits or proteasome assembly helpers cause chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures (CANDLE) or…
Abstract Number: 0796 • ACR Convergence 2023
Role of Group 2 Innate Lymphoid Cells in the Pathogenesis of Rheumatoid Arthritis
Anders Nguyen¹, Agnieszka Lastowska¹, Miriam Bollmann¹, Symeon Kourmoulakis¹, Charlotte E. van der Plas¹, Anna-Karin Hultgård Ekwall², Dietmar M. Zaiss³, Gary S Firestein⁴ and Mattias N.D Svensson⁵, ¹Department of Rheumatology and Inflammation research, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden, ²Department of Rheumatology and Inflammation research, Sahlgrenska Academy, University of Gothenburg, Kullavik, Sweden, ³Faculty of Medicine, Department of Immune Medicine, University of Regensburg, Regensburg, Germany, Regensburg, Germany, ⁴Department of Medicine, University of California San Diego, La Jolla, CA, ⁵University of Gothenburg, Gothenburg, Sweden
Background/Purpose: Immune-cell mediated activation of joint-lining fibroblast-like synoviocytes (FLS) play a key role in joint inflammation and destruction during Rheumatoid arthritis (RA). Thus, identifying factors…
Abstract Number: 1761 • ACR Convergence 2023
JAK/STAT Inhibition Modifies the Innate Lymphoid Cells 1 Immune Response in Patients with Rheumatoid Arthritis
Lidia La Barbera¹, Marianna Lo Pizzo¹, Chiara Rizzo¹, Leila Mohammadnezhad¹, federica Camarda¹, Francesco Ciccia² and Giuliana Guggino¹, ¹University of Palermo, Palermo, Italy, ²University of Campania - Luigi Vanvitelli, Naples, Italy
Background/Purpose: Recent evidence suggests that innate lymphoid cells (ILCs) might be involved in rheumatoid arthritis (RA) pathogenesis and individuals at risk of RA exhibited an…

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