Abstracts tagged "IgG4 Related Disease"

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…
Abstract Number: 0128 • ACR Convergence 2022
Advantages of Alternate-Day Glucocorticoid Treatment Strategy for IgG4-Related Disease: A Preliminary Retrospective Study ID: 1287015
Sho Fukui¹, Takehiro Nakai², Satoshi Kawaai³, Yukihiko Ikeda², Masei Suda⁴, Atsushi Nomura⁵, Hiromichi Tamaki², Mitsumasa Kishimoto⁶, Sachiko Ohde⁷ and Masato Okada², ¹Kyorin University Hospital, Tokyo, Japan, ²St. Luke's International Hospital, Tokyo, Japan, ³St. Luke's International Hospital, Chuo-ku, Tokyo, Japan, ⁴Suwa Central Hospital, Nagano, Japan, ⁵St. Luke's International Hospital, Nagareyama, Japan, ⁶Kyorin University School of Medicine, Yokohama, Japan, ⁷Center for Clinical Epidemiology, St.Luke's International University, Tokyo, Japan
Background/Purpose: Alternate-day glucocorticoid (GC) treatment is an effective treatment option that can reduce GC-associated adverse events in patients with rheumatoid arthritis and other autoimmune rheumatic…
Abstract Number: 0144 • ACR Convergence 2022
The Association of Hypocomplementemia with Organ Involvement and Serum IgG4 in IgG4-related Disease
Guy Katz¹, Amelia S. Cogan¹, Grace McMahon¹, Sebastian Perez-Espina¹, Ana Fernandes¹, Cory Perugino¹, Zachary Wallace¹, John Atkinson², Alfred Kim² and John Stone³, ¹Massachusetts General Hospital, Boston, MA, ²Washington University School of Medicine, St. Louis, MO, ³Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that can affect nearly every organ in the body. Hypocomplementemia is common in some patients with…
Abstract Number: 0147 • ACR Convergence 2022
Rituximab in IgG4-Related Diseae: An Open-Label Non-Randomized Observational Study
Jens Vikse¹, Oyvind Midtvedt², Oyvind Molberg³, Bjørg Tilde Svanes Fevang⁴, Oyvind Palm², Torhild Garen², Katrine Brække Norheim⁵, Gunnstein Bakland⁶, Marianne Wallenius⁷ and Anna-Maria Hoffmann-Vold², ¹University of Bergen, Stavanger, Norway, ²Oslo University Hospital, Oslo, Norway, ³Oslo University Hospital, Oslo, Nepal, ⁴Haukeland University Hospital, Bergen, Norway, ⁵Stavanger University Hospital, Stavanger, Norway, ⁶University Hospital of North-Norway, Tromsø, Norway, ⁷St. Olav University Hospital, Trondheim, Norway
Background/Purpose: IgG4-related disease (IgG4-RD) is a rare, heterogenous and potentially severe disease. An open-label trial demonstrated efficacy of rituximab (RTX) in IgG4-RD. Recently, four distinct…

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