Abstracts tagged "IgG4 Related Disease"

Abstract Number: 2051 • ACR Convergence 2024
Clinical Outcomes and Predictors of Relapse in Patients with IgG4-Related Disease Treated with Rituximab: A Real-World Experience
Guy Katz¹, Zachary Wallace², Grace McMahon¹, Isha Jha¹, Ana Fernandes¹, Bohang Jiang¹, Yuqing Zhang³, Hyon K. Choi⁴, Cory Perugino¹ and John Stone⁵, ¹Massachusetts General Hospital, Boston, MA, ²Massachusetts General Hospital, Newton, MA, ³Massachusetts General Hospital, Quincy, MA, ⁴Massachusetts General Hospital, Lexington, MA, ⁵Massachusetts General Hospital , Harvard Medical School, Concord, MA
Background/Purpose: Rituximab (RTX) is often used to treat IgG4-related disease (IgG4-RD), but real-world data on clinical outcomes remain limited. Defining time to relapse and predictors…
Abstract Number: 0289 • ACR Convergence 2024
Effectiveness of Rituximab in IgG4 Related Disease
Fernando Lopez-Gutierrez¹, Javier Loricera², Cristina Hormigos³, Dalifer Freites Nuñez⁴, Maria Rodriguez-Laguna⁵, Patricia Moya Albarado⁶, Marta López I Gómez⁷, Hèctor Corominas⁸, Maite Silva-Díaz⁹, GUILLERMO GONZALEZ ARRIBAS⁹, Angel Garcia-Aparicio¹⁰, Judit Font Urgelles¹¹, Ivette Casafont-Sole¹¹, Pablo Martínez Calabuig¹², Elisabet Castaneda¹³, Carolina Merino¹⁴, Raquel Zas¹⁵, Juan Molina-Collada¹⁶, Rafael Benito Melero-Gonzalez¹⁷, Eva Galindez-Agirregoikoa¹⁸, Andrea Hernández-Martín¹⁹, Lucia Pantoja²⁰, Ignacio Brana Abascal²¹, Vega Jovani²², Elia Valls-Pascual²³, Natalia Mena Vázquez²⁴, Adela Gallego- Flores²⁵, Noelia Cabaleiro Raña²⁶, Raul Veroz²⁷, Mariano Andres²⁸, Santos Castañeda²⁹ and Ricardo Blanco-Alonso³⁰, and Rituximab in IgG4 Related Disease Spanish Cooperative Group, ¹Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Cantabria, Spain, ²Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ³Hospital Clínico San Carlos, Madrid, Madrid, Spain, ⁴Hospital Clínico San Carlos. Madrid. Spain., Madrid, Spain, ⁵Resident in Rheumatology, Madrid, Spain, ⁶Hospital de San Pau, Barcelona, Spain, ⁷Hospital Universitario Alava, Vitoria, Pais Vasco, Spain, ⁸Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, ⁹Complejo Hospitalario Universitario de A Coruña, A Coruña, Spain, ¹⁰Hospital Universitario de Toledo, Toledo, Spain, ¹¹Hospital Universitari Germans Trias i Pujol, Badalona, Spain, ¹²HOSPITAL GENERAL UNIVERSITARIO VALENCIA SPAIN, Ontinyent, Comunidad Valenciana, Spain, ¹³Hospital Universitario Infanta Sofía, Madrid, Madrid, Spain, ¹⁴Hospital Universitario Puerta de Hierro Majadahonda., Majadahonda (Madrid), Spain, ¹⁵Hospital Universitario 12 de Octubre, Madrid, Madrid, Spain, ¹⁶Hospital General Universitario Gregorio Marañón, Madrid, Spain, ¹⁷CHU Ourense, O Carballino, Spain, ¹⁸BASURTO UNIVERSITY HOSPITAL, BILBAO, Spain, ¹⁹Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas, Spain, ²⁰Complejo Hospitalario Segovia, Segovia, Castilla y Leon, Spain, ²¹Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain, ²²National Health system, Alicante, Spain, ²³Hospital General de Valencia, Valencia, Comunidad Valenciana, Spain, ²⁴IBIMA, Málaga, Andalucia, Spain, ²⁵Hospital Perpetuo Socorro, Badajoz, Spain, ²⁶Hospital Universitario Montecelo, Pontevedra, Galicia, Spain, ²⁷Hospital de Merida, Merida, Extremadura, Spain, ²⁸Hospital General Universitario de Alicante, Alicante, Spain, ²⁹Hospital Universitario de la Princesa, Madrid, Spain, ³⁰Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain
Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease often associated with elevated serum IgG4 levels. High dose corticosteroids are the cornerstone of treatment, but…
Abstract Number: 2502 • ACR Convergence 2024
Characterization of the Tissue Fibroblast Repertoire in IgG4-Related Ophthalmic Disease
Eduardo Martin Nares, Gabriela Hernández-Molina, Diana Aguilar-León, Norma Ofelia Uribe-Uribe and Janette Furuzawa-Carballeda, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by tissue infiltration with IgG4+ plasma cells. Fibrosis in the target organs is one of…
Abstract Number: L16 • ACR Convergence 2023
Withdrawal of Immunosuppressant and Low-dose Steroids in IgG4-RD Patients with Stable Disease (WInS IgG4-RD): An Investigator-initiated, Multi-center, Open-label, Randomized Controlled Trial
Linyi Peng¹, Yuxue nie¹, Jiaxin Zhou¹, Lijun Wu², Fang Wang³, Xiaomei Chen⁴, Jieqiong Li¹, Yu Peng¹, Hui Lu¹, Lidan Zhao¹, Mengtao Li¹, Yan Zhao¹, Xiaofeng Zeng⁵, Yunyun Fei¹ and Wen Zhang¹, ¹Peking Union Medical College Hospital，Department of Rheumatology and Clinical Immunology, National Clinical Research Center for Dermatologic and Immunologic Diseases, State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China, ²Xinjiang Uygur Autonomous Region People's Hospital, XinJiang, China, ³Beijing Hospital, Beijing, China, ⁴Xinjiang Uygur Autonomous Region People's Hospital, Wulumuqi, China, ⁵Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China
Background/Purpose: IgG4-related disease (IgG4-RD) is a fibroinflammatory disease. Remission induction treatment with glucocorticoid (GC) is usually effective, but its tendency of relapse makes the strategy…
Abstract Number: 0715 • ACR Convergence 2023
Glucocorticoid Toxicity in Patients with IgG4-Related Disease Within the First Year of Treatment
Ana Mora-Rosas, Gabriela Hernandez-Molina, Edwin Ulises Cortez-Domínguez and Eduardo Martin-Nares, Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
Background/Purpose: Patients with IgG4-related disease (IgG4-RD) often require long-term glucocorticoid therapy. The prevalence, types, and underlying factors of glucocorticoid toxicity in IgG4-RD patients remain unknown.…
Abstract Number: 0716 • ACR Convergence 2023
Efficacy of Immunosuppressants for Disease Relapse in Patients with IgG4-related Disease at Our Institution
Kanako Chujo¹, Hiromi Shimada², Hiroki Ozaki³, Risa Wakiya⁴, Shusaku Nakashima⁴, taichi miyagi⁵, Yusuke Ushio⁶, Koichi Sugihara⁷, Mao Mizusaki⁴, Rina Mino⁸, Ryoko Kagawa⁹, Hayamasa Yamaguchi⁹, Tomohiro Kameda² and Hiroaki Dobashi², ¹Kagawa University, Miki, Kita District, Kagawa, Japan, ²Kagawa University, Kagawa, Japan, ³Department of Rheumatology, KKR Takamatsu Hospital, Kagawa, Japan, ⁴Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kagawa, Japan, ⁵Kagawa university, Kidagun, Japan, ⁶Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Miki, Kita District, Kagawa, Japan, ⁷Kagawa University, Miki-cho, Kita-gun, Japan, ⁸Kagawa University, Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa, Japan, ⁹Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Miki-cho, Kita-gun, Japan
Background/Purpose: Most patients with IgG4-related disease (IgG4-RD) have a good response to glucocorticoid (GC) therapy, however, disease relapses sometimes occur during GC tapering. Therefore, the…
Abstract Number: 1023 • ACR Convergence 2023
Characterization of Phenotypic Differences in IgG4-Related Disease Across the Sexes
Grace McMahon¹, Isha Jha¹, Aubree McMahon¹, Ana Fernandes¹, Zachary Wallace², Guy Katz¹, Cory Perugino¹ and John Stone³, ¹Massachusetts General Hospital, Boston, MA, ²Massachusetts General Hospital, Newton, MA, ³Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Concord, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is a multi-organ fibroinflammatory disease with an autoimmune basis that can affect essentially any organ. Differences in phenotypic expression between males…
Abstract Number: 1131 • ACR Convergence 2023
Clinical Phenotypes of IgG4-related Disease in a Multi-ethnic Singapore Cohort
Choon Guan Chua¹, Weida Chew¹, Jeggrey Kay Wee Kam¹, Hwee-Pin Phua¹, Chengzi Chow¹, Carol Yee-Leng Ng¹, Wei-Yen Lim¹, Ru Sin Lim¹, Jereme Yijin Gan¹, Mung-Ee Loh¹, Tianrong Yeo², David Zhiwei Law¹, Stephrene Seok Wei Chan³, Amanda Francesca Ya-Fang Cheang⁴, Yong-Howe Ho¹ and Charles Kien-Fong Vu¹, ¹Tan Tock Seng Hospital, Singapore, Singapore, ²National Neurology Institute, Singapore, Singapore, ³National University Hospital, Singapore, Singapore, ⁴Woodlands Health Campus, Singapore, Singapore
Background/Purpose: IgG4-related disease (IgG4-RD) is an immune-mediated condition that is heterogenous and can result in organ failure. To understand the disease profile of our local…
Abstract Number: 0002 • ACR Convergence 2023
Population Pharmacokinetic and Pharmacodynamic Analyses of Obexelimab in Healthy Volunteers and in Patients with Rheumatoid Arthritis or IgG4-Related Diseases
Xiaodong Wang, Rachel Kirk, Simon Lowry and Hua Mu, Zenas BioPharma, Waltham, MA
Background/Purpose: Obexelimab is a novel bifunctional antibody that inhibits B-cells, CD19-expressing plasma cells, and plasmablast activity and has the potential to provide clinical benefits across…
Abstract Number: 1154 • ACR Convergence 2023
Clinical Presentation of IgG4-Related Disease. Single Referral Hospital Experience and Literature Review
Fernando López¹, Javier Loricera² and Ricardo Blanco³, ¹Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ²Hospital Universitario Marqués de Valdecilla, Santander, Spain, ³Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
Background/Purpose: IgG4-related disease (IgG4-RD) is an inflammatory and fibrosing entity with very heterogeneous clinical manifestations. It was recognized as a new disease entity only 12…
Abstract Number: 0003 • ACR Convergence 2023
Expanded Extrafollicular B Cells Were Improved by RTX in IgG4-related Disease
Yusho Ishii¹, Aakriti Alisha Arora¹, Scott Jenks¹, Iñaki Sanz² and Arezou Khosroshahi¹, ¹Emory University, Atlanta, GA, ²Emory University School of Medicine, Atlanta, GA
Background/Purpose: IgG4-related disease (IgG4-RD) is an immune-mediated disease characterized by fibrotic masses with expansion of IgG4-producing plasma cell in multiple organs such as pancreas, lacrimal…
Abstract Number: 1863 • ACR Convergence 2023
Immune-mediated Hypertrophic Pachymeningitis: Focusing on the Localization and Volume of Thickened Dura Mater Lesion
Yasuhiro Shimojima¹, Junji Ikeda² and Yoshiki Sekijima¹, ¹Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan, ²Department of Medicine (Neurologu and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan
Background/Purpose: Hypertrophic pachymeningitis (HP) is a rare inflammatory neurological disorder characterized by the thickened dura mater with extensive tissue fibrosis and immune-mediated inflammation. Notably, headaches…
Abstract Number: 0005 • ACR Convergence 2023
Obexelimab Inhibits B Cell Activation and May Interfere with B Cell Chemotaxis in IgG4-Related Disease
Thomas Guy¹, Hang Liu², Vinay Mahajan², Cory Perugino³, Zachary Wallace⁴, Shauna quinn⁵, Allen Poma⁶, Debra Zach⁷, John Stone⁸ and Shiv Pillai⁹, ¹Ragon Institute of MGH, MIT and Harvard, Cambridge, MA, ²Ragon Institute, Cambridge, MA, ³Massachusetts General Hospital, Boston, MA, ⁴Massachusetts General Hospital, Newton, MA, ⁵Zenas Biopharma, New York, NY, ⁶Zenas Biosciences, New York, NY, ⁷Exagen, Vista, CA, ⁸Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Concord, MA, ⁹Harvard Medical School, Boston, MA
Background/Purpose: Obexelimab is a bifunctional, non-cytolytic, humanized monoclonal antibody that binds CD19 and ligates FcγRIIb with high affinity. Data from a phase 2 clinical trial…
Abstract Number: 1917 • ACR Convergence 2023
Reducing Inadvertent Antifilarial Antibody Testing at an Academic Medical Center: A Quality Improvement Project
Khiem Vu, Justin Choi and Jon Golenbiewski, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem, NC
Background/Purpose: IgG4-Related Disease (IgG4-RD) is a fibroinflammatory autoimmune disease that can present with glandular swelling, chronic pancreatitis, sclerosing cholangitis, and retroperitoneal fibrosis, amongst others. Along…
Abstract Number: 0030 • ACR Convergence 2023
A Pathogenetic Model of IgG4-related Disease Developed from Familial IKZF1 and UBR4 Gene Variants
Qingxiang Liu¹, Kenneth Warrington¹, Matthew Koster¹, Cornelia M. Weyand² and Jorg Goronzy¹, ¹Mayo Clinic, Rochester, MN, ²Mayo Clinic School of Medicine and Stanford University, Rochester, MN
Background/Purpose: Most autoimmune diseases are polygenic, frequently with more than 100 gene variants contributing to genetic predisposition. Given this complexity, conclusions on disease mechanisms are…

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