Abstracts tagged "IgG4 Related Disease"

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: 2529 • ACR Convergence 2025
IgG4-Related Disease Paravertebral and Ureteropelvic Mass Presentations are Linked to Specific Disease Features
Federica Bonaso¹, Grace McMahon², Isha Jha², Guy Katz², Zachary Wallace³, John Stone⁴ and Cory Perugino⁵, ¹University of Brescia, ASST Spedali Civili of Brescia, Massachusetts General Hospital, Monza, Italy, ²Massachusetts General Hospital, Boston, MA, ³Massachusetts General Hospital, Newton, MA, ⁴Massachusetts General Hospital , Harvard Medical School, Concord, MA, ⁵Massachusetts General Hospital, Harvard Medical School, Boston, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is a chronic immune-mediated disease characterized by mass-forming fibrotic lesions. The aim of this work was to characterize the overall clinical…
Abstract Number: 0992 • ACR Convergence 2025
FoxP3Lo CD4+ T cells are functionally suppressive and expanded in the immune-mediated fibrotic diseases IgG4-related disease and systemic sclerosis
Laura J. Yockey¹, Ian Doyle², Thomas Guy³, Devanshi Trivedi², Chinmay Gadiraju⁴, Federica Bonaso⁵, Jesse Akaa², Alison Puri⁶, Zachary Wallace⁷, Guy Katz⁸, Sydney Montesi⁸, John Stone⁹, Flavia Castelino⁸, Shiv Pillai¹⁰, Andrew Luster¹¹, Vinay S. Mahajan¹² and Cory Perugino¹³, ¹MGH, Charlestown, MA, ²Massachusetts General Hospital (MGH - Mass General) (MGB), Boston, MA, ³Ragon Institute of MGH, MIT and Harvard/ Royal Prince Alfred Hospital Sydney, Sydney, Australia, ⁴Ragon Institute of MGH, MIT and Harvard, Cambridge, MA, ⁵University of Brescia, ASST Spedali Civili of Brescia, Massachusetts General Hospital, Monza, Italy, ⁶Boston University, Brookline, MA, ⁷Massachusetts General Hospital, Newton, MA, ⁸Massachusetts General Hospital, Boston, MA, ⁹Massachusetts General Hospital , Harvard Medical School, Concord, MA, ¹⁰Harvard Medical School, Cambridge, MA, ¹¹Massachusetts General Hospital, Charlestown, MA, ¹²Brigham and Women's Hospital, Boston, MA, ¹³Massachusetts General Hospital, Harvard Medical School, Boston, MA
Background/Purpose: The absence of regulatory T cells (Tregs) results in multiorgan autoimmunity in the context of monogenic “Tregopathies,” but their role in mediating polygenic autoimmune…
Abstract Number: 2528 • ACR Convergence 2025
Broad Screening of the Human Proteome Identifies a Cassette of Six Autoantigens that Distinguishes IgG4-Related Disease
Federica Bonaso¹, Zhenrui Zhang², Mahilan Guha³, Ian Doyle⁴, Jesse Akaa⁴, Grace McMahon⁵, Isha Jha⁵, Sydney Montesi⁵, Thomas Guy⁶, Guy Katz⁵, Zachary Wallace⁷, John Stone⁸, Vinay S. Mahajan⁹, Shiv Pillai¹⁰ and Cory Perugino¹¹, ¹University of Brescia, ASST Spedali Civili of Brescia, Massachusetts General Hospital, Monza, Italy, ²Ragon Institute of Mass General Brigham, MIT, and Harvard, Boston, ³Montgomery Blair High School, Brigham and Women’s Hospital, Boston, ⁴Massachusetts General Hospital (MGH - Mass General) (MGB), Boston, MA, ⁵Massachusetts General Hospital, Boston, MA, ⁶Ragon Institute of MGH, MIT and Harvard/ Royal Prince Alfred Hospital Sydney, Sydney, Australia, ⁷Massachusetts General Hospital, Newton, MA, ⁸Massachusetts General Hospital , Harvard Medical School, Concord, MA, ⁹Brigham and Women's Hospital, Boston, MA, ¹⁰Harvard Medical School, Cambridge, MA, ¹¹Massachusetts General Hospital, Harvard Medical School, Boston, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is a chronic immune-mediated disease typified by mass-forming lesions. Self-antigens driving the oligoclonal expansion of plasmablasts have previously been reported. However,…
Abstract Number: 0910 • ACR Convergence 2025
Development of Four Distinct Human IgG4-Producing Mouse Models Recapitulating IgG4-Related Disease
MIN GANG KIM¹, JINA YEO², HEA RIM KANG³, JAE HYUN MOON³, Seon Uk Kim⁴, SeoYoon Ban⁵, MI HYEON KIM⁶ and Eun Young Lee⁷, ¹Seoul National University College of Medicine, Seoul, Republic of Korea, ²Division of Rheumatology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Republic of Korea, ³Seoul National University College of Medicine, Jongno-gu, Seoul, Republic of Korea, ⁴Seoul National University, Jongno-gu, Seoul, Republic of Korea, ⁵Department of Cancer biology, Graduate School of College of Medicine, Seoul National University, Korea, Seoul, Republic of Korea, ⁶Hallym university dongtan sacred heart hospital, Seocho-gu, Seoul, Republic of Korea, ⁷Seoul National University College of Medicine, Seoul, South Korea
Background/Purpose: IgG4-related disease (IgG4RD) is an immune-mediated fibroinflammatory condition characterized by multi-organ involvement, elevated serum IgG4, and IgG4-positive plasma cell infiltrates that form tumor-like lesions…
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,…
Abstract Number: 1675 • ACR Convergence 2024
Extended Validation of the IgG4-Related Disease Symptom Severity Index (SSI)
Isha Jha¹, Grace McMahon¹, Guy Katz¹, Shruthi Srivatsan¹, Caleb Bolden¹, Ana Fernandes¹, Cory Perugino¹, John Stone² and Zachary Wallace³, ¹Massachusetts General Hospital, Boston, MA, ²Massachusetts General Hospital , Harvard Medical School, Concord, MA, ³Massachusetts General Hospital, Newton, MA
Background/Purpose: The Symptom Severity Index (SSI) is a patient-reported outcome measure (PROM) developed for IgG4-related disease (IgG4-RD). The SSI assesses symptoms of IgG4-RD and the…
Abstract Number: 1835 • ACR Convergence 2024
FoxP3Hi CTLA4+ ICOS+ Regulatory T Cells Are Expanded in Patients with Sarcoidosis but Not Systemic Sclerosis or IgG4-Related Disease
Laura Yockey¹, Thomas Guy², Chinmay Gadiraju³, Ian Doyle¹, Jesse Akaa⁴, Alison Puri⁵, Zachary Wallace⁶, Guy Katz¹, Sydney Montesi¹, John Stone⁷, Flavia Castelino¹, Shiv Pillai⁸, Andrew Luster⁹, Vinay Mahajan³ and Cory Perugino¹, ¹Massachusetts General Hospital, Boston, MA, ²Ragon Institute of MGH, MIT and Harvard, Cambridge, MA, ³Brigham and Women's Hospital, Boston, MA, ⁴Massachussets General Hospital, Boston, MA, ⁵Boston University, Brookline, MA, ⁶Massachusetts General Hospital, Newton, MA, ⁷Massachusetts General Hospital , Harvard Medical School, Concord, MA, ⁸Harvard Medical School, Cambridge, MA, ⁹Massachusetts General Hospital, Charlestown, MA
Background/Purpose: The absence of regulatory T cells (Tregs) results in multiorgan autoimmunity in the context of monogenic “Tregopathies”, but their role in mediating polygenic autoimmune…

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