Abstracts tagged "IgG4 Related Disease"

Abstract Number: 0706 • ACR Convergence 2023
Aortitis and Periaortitis Spectrum in 135 Patients from a Single Referral Centre
Fernando López¹, Javier Loricera², Carmen Secada³, Lucía García-Alcalde⁴, María Núñez-Savar⁴, Ander Ucelay-Aristi⁴ and Ricardo Blanco⁵, ¹Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ²Hospital Universitario Marqués de Valdecilla, Santander, Spain, ³Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain, ⁴Department of Cardiovascular Surgery, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ⁵Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
Background/Purpose: Aortitis is the inflammation of the aortic wall, and can be idiopathic or associated with a cluster of infectious and non-infectious diseases (1-3). Periaortitis,…
Abstract Number: 0713 • ACR Convergence 2023
IgG4-Related Disease as a Potencial Etiology of Idiopathic Constrictive Pericarditis
Edwin Ulises Cortez-Domínguez¹, Gabriela Hernandez-Molina¹, Norma Ofelia Uribe-Uribe², María J Lizardo-Thiebaud² and Eduardo Martin-Nares¹, ¹Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ²Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
Background/Purpose: Constrictive pericarditis (CP) is a type of diastolic heart failure characterized by impaired cardiac filling due to the presence of an inelastic pericardium. Histologically,…
Abstract Number: 0714 • ACR Convergence 2023
Single Center Experience of Rituximab Treatment for IgG4-RD Disease in a Large Multi-ethnic Cohort
Matthew Colquhoun¹, Taryn Youngstein² and James Tomlinson³, ¹Department of Rheumatology, Imperial College Healthcare Trust, London, United Kingdom, ²National Heart & Lung Institute, Imperial College London, London, United Kingdom, ³Department of nephrology, Imperial College Healthcare Trust, London, United Kingdom
Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disorder that can affect almost any organ system. Response to corticosteroids(CS) is typically prompt although adverse effects…
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…
Abstract Number: 1933 • ACR Convergence 2023
Analysis of IgG4-Related Disease in a Diverse Long Island Population: Insights from the Rheumatology Clinic
Young Min Cho¹, Galina Marder², Koushik Sangaraju³, Lara El Khoury², Kourosh Shargani⁴ and Jevon Fragoso⁵, ¹Northwell, Glen Cove, NY, ²Northwell, Great Neck, NY, ³Staten Island University Hospital - Northwell Health, Staten Island, NY, ⁴Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Manhasset, NY, ⁵Northwell, Queens, NY
Background/Purpose: IgG4-related disease (IgG4RD) is an immune-mediated disease characterized by dense lymphoid-lymphoplasmacytic infiltrates with high IgG4-positive plasma cells with heterogeneous clinical manifestations. The data on…

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Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. Academic institutions, private organizations and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part a scientific presentation or presentation of additional new information that will be available at the time of the meeting) is under embargo until Saturday, November 11, 2023.

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