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Abstracts tagged "IgG4 Related Disease"

  • Abstract Number: 0032 • ACR Convergence 2025

    Protein Language Model-Guided Homology Identifies Microbial Enzymes Linked to Fibrosis-Prone IgG4-RD and Crohn’s Disease

    Kumar Thurimella1, Ahmed Mohamed2, Chenhao Li3, Tommi Vatanen4, Daniel Graham3, Roisin Owens5, Sabina Leanti La Rosa6, Damian Plichta3, Sergio Bacallado5 and Ramnik Xavier7, 1University of Colorado School of Medicine, Aurora, CO, 2Broad Institute, Boston, 3Broad Institute, Cambridge, MA, 4University of Helsinki, Helsinki, Finland, 5University of Cambridge, Cambridge, United Kingdom, 6NMBU, As, Norway, 7Harvard Medical School, Boston, MA

    Background/Purpose: Uncharacterized microbial enzymes in metagenomics are difficult to annotate, especially in fibrosis-prone conditions like IgG4-related disease (IgG4-RD) and Crohn’s disease (CD), where microbial carbohydrate…
  • Abstract Number: 1666 • ACR Convergence 2025

    Treatment with Rilzabrutinib was associated with Rapid and Sustained Reduction in Disease Activity in Patients with IgG4-RD Previously Treated or Naïve to B cell Depletion

    John Stone1, Mollie Carruthers2, Alireza Meysami3, Matthew Charles Baker4, Daniela Ghetie5, Lindsay Lally6, Fernando Martinez-Valle7, Lorenzo Dagna8, nicolas schleinitz9, Jeea Choi10, Leda Mannent11 and Owen Hagino10, 1Massachusetts General Hospital , Harvard Medical School, Concord, MA, 2Vancouver General Hospital, Vancouver, BC, Canada, 3Henry Ford Health, Detroit, MI, 4Stanford University, Menlo Park, CA, 5Oregon Health and Science University, Portland, OR, 6Hospital for Special Surgery, NEW YORK, NY, 7Vall d’Hebron Hospital, Barcelona, Spain, 8IIRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, 9Aix Marseille university, AP-HM, Marseille, France, 10Sanofi, Morristown, NJ, 11Sanofi, Gentilly, France

    Background/Purpose: IgG4-RD is a progressive, immune-mediated fibrotic disease with limited treatment options. Rilzabrutinib is a reversible, orally available inhibitor of Bruton's tyrosine kinase (BTKi). Here,…
  • Abstract Number: 1190 • ACR Convergence 2025

    IgG4-related disease with comorbid autoimmune diseases: a retrospective study from a large cohort

    Xinli Yang1, Wen Zhang2, Jiaxin zhou3 and Yuxue Nie4, 1Peking Union Medical College, Dong Cheng Qu, China (People's Republic), 2Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), 3Peking Union Medical College Hospital, Beijing, China (People's Republic), 4Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China, Dong Cheng Qu, China (People's Republic)

    Background/Purpose: This study aimed to explore the clinical characteristics, treatment, and prognosis of IgG4-related disease (IgG4-RD) patients with comorbid autoimmune diseases (AIDs).Methods: A total of…
  • Abstract Number: 1173 • ACR Convergence 2025

    Prevalence of Coronary Artery Involvement in IgG4-Related Disease Detected by Non-Gated Cross-Sectional Imaging

    Matthew Charles Baker1, Lauren Taylor2, Haziq Siddiqi2, Jison Hong2 and Brian Pogatchnik2, 1Stanford University, Menlo Park, CA, 2Stanford University, Stanford, CA

    Background/Purpose: IgG4-related disease (IgG4-RD) classically presents with a mass or diffusely enlarged organ, but it also affects blood vessels of all sizes. Coronary artery involvement…
  • Abstract Number: 1172 • ACR Convergence 2025

    A Phase II, Single-Site, Open-Label Study of Zanubrutinib in Patients with IgG4-Related Disease

    Matthew Charles Baker1, Audra Horomanski2, Robert Fairchild3, Yuhan Liu3, Mariani Diaz Deluna3, Tobias Lanz4, Saurabh Gawde3, Mehdi Khalighi3, Benjamin Franc3, Mrudula Penta3, Nancy Pham3 and Kip Guja3, 1Stanford University, Menlo Park, CA, 2Stanford University, Palo Alto, CA, 3Stanford University, Stanford, CA, 4Stanford University, Stanford

    Background/Purpose: IgG4-related disease (IgG4-RD) is a chronic, systemic, immune-mediated disease that is commonly treated with B cell depletion. However, due to potential risks associated with…
  • Abstract Number: 1167 • ACR Convergence 2025

    Clinical Characteristics and Outcomes of IgG4-Related Disease Patients Who Chose Watchful Observation without Treatment Intervention: A Single-Center Study

    Kanako Chujo1, Hiromi Shimada2, Shusaku Nakashima2, Taichi Miyagi1, Koichi Sugihara2, Yusuke Ushio1, Mao Mizusaki2, Naoto Manabe1, Mayuko Wada2, Risa Wakiya3, Hiroki Ozaki4 and Hiroaki Dobashi2, 1Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Miki-cho, Kita-gun, Japan, 2Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Miki-cho, Kita-gun, Kagawa, Japan, 3Division of Rheumatology, Departent of Internal Medicine, Toho University School of Medicine, Tokyo, Japan, 4KKR Takamatsu Hospital, Takamatsu, Kagawa, Japan

    Background/Purpose: Treatment of IgG4-related disease (IgG4-RD) is recommended for patients with severe organ involvement or for patients with reduced quality of life even in the…
  • Abstract Number: 2546 • ACR Convergence 2025

    Diagnostic Journey, Clinical Burden, And Quality Of Life Of Patients With IGg4-Related Disease: Results Of A Cross-Sectional Survey Of Patients And Physicians In The United States

    Yasmin Hernandez-Barco1, Jenny Y. Park2, Rajvi Wani3, Andrea Meyers4, Matthew Charles Baker5, Leonard Calabrese6, Kristina Patterson2, Hannah Connolly7, Shiva Lauretta Birija7 and Emma Culver8, 1Massachusetts General Hospital; Harvard Medical School, Boston, MA, 2Amgen Inc., Thousand Oaks, CA, 3Amgen Canada Inc., Mississauga, ON, Canada, 4Amgen Canada Inc., Thousand Oaks, ON, Canada, 5Stanford University, Menlo Park, CA, 6Cleveland Clinic, Cleveland, OH, 7Adelphi Real World, Bollington, United Kingdom, 8John Radcliffe Hospital; University of Oxford, Oxford, United Kingdom

    Background/Purpose: IgG4-related disease (IgG4-RD) is a chronic immune-mediated fibroinflammatory condition characterized by fibrosis and organ damage. Although IgG4-RD is increasingly recognized, real-world clinical burden remains…
  • Abstract Number: 1163 • ACR Convergence 2025

    Clinical and Biomarker Characteristics of Inebilizumab-Treated Participants Who Experienced an IgG4-RD Flare During the Phase 3 MITIGATE Trial

    John Stone1, Emma Culver2, Arezou Khosroshahi3, Wen Zhang4, Emanuel Della Torre5, Kazuichi Okazaki6, Yoshiya Tanaka7, Matthias Lohr8, nicolas schleinitz9, Fernando Martinez-Valle10, Manu Nayar11, Vinciane Rebours12, Cory Perugino13, Kristen Clarkson14, Theresa Alexander14, Xinxin Dong14, Qian Huang14, Sue Cheng14 and Daniel Cimbora14, 1Massachusetts General Hospital , Harvard Medical School, Concord, MA, 2John Radcliffe Hospital; University of Oxford, Oxford, United Kingdom, 3Emory University, Atlanta, GA, 4Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), 5IRCCS San Raffaele Scientific Institute, Milan, Italy, 6Kansai Medical University Kori Hospital, Osaka, Japan, 7University of Occupational and Environmental Health, Japan, Kitakyushu, Japan, 8Karolinska Institutet, Stockholm, Sweden, 9Aix Marseille university, AP-HM, Marseille, France, 10Vall d’Hebron Hospital, Barcelona, Spain, 11Freeman Hospital, Newcastle upon Tyne, United Kingdom, 12Beaujon Hospital, AP-HP, Université Paris-Cité, Clichy, France, 13Massachusetts General Hospital, Harvard Medical School, Boston, MA, 14Amgen, Thousand Oaks, CA

    Background/Purpose: IgG4-RD is a rare, chronic, fibroinflammatory disorder characterized by recurrent flares that can affect any organ and lead to permanent tissue damage. Inebilizumab (INEB)…
  • Abstract Number: 2531 • ACR Convergence 2025

    Coronary periarteritis in IgG4-Related Disease

    Georges El Hasbani1, Daniel Larson2, Matthew Koster1 and Kenneth Warrington1, 1Mayo Clinic, Rochester, MN, 2Mayo Clinic, Rochester

    Background/Purpose: IgG4-related disease (IgG4-RD) is characterized by chronic inflammation and fibrosis, which can affect multiple organs, especially the pancreas, salivary glands, and retroperitoneum (1). It…
  • Abstract Number: 1154 • ACR Convergence 2025

    Assessment Of Damage Using The IgG4-Related Disease Damage Index.

    Eduardo Martin-Nares1 and Gabriela Hernandez-Molina2, 1Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, 2Instituto Nacional de Ciencias Medicas y Nutricion, Mexico City, Federal District, Mexico

    Background/Purpose: IgG4-related disease (IgG4-RD) is a chronic fibroinflammatory disorder affecting multiple organs, often leading to irreversible damage. Standardized tools like the IgG4-RD Damage Index (DI)…
  • Abstract Number: 2530 • ACR Convergence 2025

    Broad Screening of Inflammation-Associated Proteins Identifies Serum CCL19 and CCL2 as Versatile Biomarkers of Disease Activity in IgG4-Related Disease

    Federica Bonaso1, Bohang Jiang2, Laura J. Yockey3, Jesse Akaa4, Ian Doyle4, Evan Hsu5, Courtney Leson6, Grace McMahon7, Isha Jha7, Sydney Montesi7, Guy Katz7, Shiv Pillai8, Pui Lee5, John Stone9, Zachary Wallace10 and Cory Perugino11, 1University of Brescia, ASST Spedali Civili of Brescia, Massachusetts General Hospital, Monza, Italy, 2MGH, Boston, MA, 3MGH, Charlestown, MA, 4Massachusetts General Hospital (MGH - Mass General) (MGB), Boston, MA, 5Boston Children's Hospital, Newton, MA, 6Boston Children's Hospital, Boston, 7Massachusetts General Hospital, Boston, MA, 8Harvard Medical School, Cambridge, MA, 9Massachusetts General Hospital , Harvard Medical School, Concord, MA, 10Massachusetts General Hospital, Newton, MA, 11Massachusetts General Hospital, Harvard Medical School, Boston, MA

    Background/Purpose: IgG4-related disease (IgG4-RD) is a chronic immune-mediated disease characterized by mass-forming lesions and organ dysfunction. The smoldering tempo and often asymptomatic nature of IgG4-RD…
  • Abstract Number: 1036 • ACR Convergence 2025

    Factors Associated With Long-term Remission After Drug Discontinuation In IgG4-related Disease: A 3-Year Rule

    Yifei Wang1, Yuxue Nie2, Jingna Li3, Jialei Zhang4, Qinhuan Luo1, Xinli Yang5, Jialing Jiang1, Nianyi Zhang6, Linyi Peng7, Yunyun Fei6, Jiaxin zhou4 and Wen Zhang8, 1Peking Union Medical College, Beijing, China (People's Republic), 2Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China, Dong Cheng Qu, China (People's Republic), 3Peking Union Medical College, Dong Cheng Qu, Beijing, China, 4Peking Union Medical College Hospital, Beijing, China (People's Republic), 5Peking Union Medical College, Dong Cheng Qu, China (People's Republic), 6Peking Union Medical College Hospital, Beijing, Beijing, China (People's Republic), 7PEKING UNION MEDICAL COLLEGE HOSPITAL, Beijing, Beijing, China, 8Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic)

    Background/Purpose: IgG4-related disease (IgG4-RD) is a chronic fibroinflammatory disorder with multi-organ involvement and elevated serum IgG4 levels. Glucocorticoids (GC) with or without immunosuppressants (IM) are…
  • Abstract Number: 0775 • ACR Convergence 2024

    A Phase 3, Randomized, Double-Blind, Multicenter, Placebo-Controlled Study of Inebilizumab in IgG4-Related Disease (MITIGATE): Primary Efficacy and Safety Findings

    John Stone1, Emma Culver2, Arezou Khosroshahi3, Wen Zhang4, Emanuel Della Torre5, Kazuichi Okazaki6, Yoshiya Tanaka7, Matthias Löhr8, Nicolas Schleinitz9, Lingli Dong10, Hisanori Umehara11, Marco Lanzillotta12, Zachary Wallace13, Mikael Ebbo14, George Webster15, Ferran Martinez Valle16, Manu Nayar17, Vinciane Rebours18, Cory Perugino19, Xinxin Dong20, Yanping Wu20, Nishi Rampal20 and Daniel Cimbora20, 1Massachusetts General Hospital , Harvard Medical School, Concord, MA, 2John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom, 3Emory University, Atlanta, GA, 4Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), 5Ospedale San Raffaele, Milan, Italy, 6University of Occupational and Environmental Health, Osaka, Japan, 7Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Fukuoka, Japan, 8Karolinska Institutet, Stockholm, Sweden, 9Aix Marseille university, AP-HM, Marseille, France, 10Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (People's Republic), 11Department of Rheumatology and Immunology, Nagahama City Hospital,, Nagahama, Shiga 526-0043, Japan, Japan, 12IRCCS Ospedale San Raffaele, Milano, Italy, 13Massachusetts General Hospital, Newton, MA, 14Aix-Marseille Université, Marseille, France, 15University College Hospital London, London, United Kingdom, 16Vall d’Hebron Hospital, Barcelona, Spain, 17Freeman Hospital, Newcastle Upon Tyne, United Kingdom, 18Beaujon Hospital, Université Paris-Cité, France, 19Massachusetts General Hospital, Boston, MA, 20Amgen Inc., Thousand Oaks, CA

    Background/Purpose: IgG4-related disease (IgG4-RD) is a rare, systemic, fibroinflammatory disease characterized by unpredictable and recurring flares, leading to organ damage and decreased quality of life.…
  • Abstract Number: 1113 • ACR Convergence 2024

    2nd Analysis of IgG4-Related Disease in a Diverse Long Island Population: Utilizing Serological and Histological Markers for Assessing Treatment Response

    Young Min Cho1, Alejandro Quintero Villegas2, Matt Means3, Zanos Theodoros2, Lara El Khoury4 and Galina Marder3, 1Northwell, Glen Cove, NY, 2Feinstein Institutes for Medical Research, Manhasset, NY, 3Northwell, Manhasset, NY, 4Division of Rheumatology/Northwell Health, Great Neck, NY

    Background/Purpose: IgG4-RD presents various clinical manifestations, making it challenging to assess treatment responses.  B-cell depletion agents such as Rituximab (RTX) are used in refractory cases.…
  • Abstract Number: 1123 • ACR Convergence 2024

    Serological Elevation of IgG4: Clinical Correlation and Differential Diagnosis

    Pablo Martínez Calabuig1, Laura Salvador Maicas2, Mireia Lucía Sanmartín Martínez3, Iván Jesús Lorente Betanzos3, Jorge Juan Fragío Gil4, Roxana González Mazario5, Amalia Rueda5, juan José Lerma Garrido6, Clara Molina Almela3 and Cristina Campos Fernández7, 1HOSPITAL GENERAL UNIVERSITARIO VALENCIA SPAIN, Ontinyent, Comunidad Valenciana, Spain, 2Consorcio Hospital General Universitario de Valencia, Castellón de la Plana, Comunidad Valenciana, Spain, 3Consorcio Hospital General Universitario de Valencia, Valencia, Comunidad Valenciana, Spain, 4Hospital General Universitario, Valencia, Comunidad Valenciana, Spain, 5Hospital General de Valencia, Valencia, Comunidad Valenciana, Spain, 6Hospital General Universitario Valencia, Valencia, Comunidad Valenciana, Spain, 7Department of Rheumatology. Hospital General Universitario de Valencia, Valencia, Spain

    Background/Purpose: IgG4-related disease (IgG4-RD) is a rare, systemic, immunomediated fibroinflammatory process with uncertain etiology and pathophysiology that can affect multiple organs, presenting common clinical, radiological,…
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