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Abstracts tagged "IgG4 Related Disease"

  • Abstract Number: 0285 • ACR Convergence 2024

    Anti-THSD7A Antibodies Are Not Broadly Associated with IgG4-Related Disease or IgG4-Related Membranous Nephropathy

    Guy Katz1, Jesse Akaa2, Grace McMahon1, Isha Jha1, Ian Doyle1, Ana Fernandes1, Zachary Wallace3, Shiv Pillai4, John Stone5 and Cory Perugino1, 1Massachusetts General Hospital, Boston, MA, 2Massachussets General Hospital, Boston, MA, 3Massachusetts General Hospital, Newton, MA, 4Harvard Medical School, Cambridge, MA, 5Massachusetts General Hospital , Harvard Medical School, Concord, MA

    Background/Purpose: Membranous nephropathy (MN) is a known manifestation of IgG4-related disease (IgG4-RD). Unlike primary MN, most patients with IgG4-related MN do not have anti-phospholipase 2…
  • Abstract Number: 2045 • ACR Convergence 2024

    Descriptive Study on the Use of Serum Levels of Immunoglobulin G4, Immunoglobulin E and Eosinophil Count as Serum Biomarkers Across Four Clinical Phenotypes of IgG4-Related Disease

    Vineetha Philip1, Soudabeh Daliri2, Alma Aveytia Camacho2, Ruba Memon3, Myriam Guevara2 and Sarah Kazzaz4, 1Houston Methodist Hospital, Sugar Land, TX, 2Houston Methodist, Houston, TX, 3Houston Methodist, Missouri City, TX, 4Houston Methodist Hospital, Houston, TX

    Background/Purpose: IgG4 – Related Disease (IgG4-RD) is a multisystem immune-mediated fibroinflammatory disorder that can affect nearly any organ and can mimic other inflammatory conditions, malignancy…
  • Abstract Number: 0286 • ACR Convergence 2024

    Damage Associated with Disease-Specific Effects and Diagnostic or Therapeutic Procedures Is Highly Prevalent in IgG4-Related Disease

    Guy Katz1, Zachary Wallace2, Cory Perugino1, Grace McMahon1, Isha Jha1, Ana Fernandes1 and John Stone3, 1Massachusetts General Hospital, Boston, MA, 2Massachusetts General Hospital, Newton, MA, 3Massachusetts General Hospital , Harvard Medical School, Concord, MA

    Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that can affect nearly any organ or anatomic site. Although the disease responds well to glucocorticoids…
  • 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…
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