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Abstracts tagged "IgG4 Related Disease"

  • 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: 0536 • ACR Convergence 2024

    A Population Modeling and Simulation of the Effect of Obexelimab Exposure on the QTc Interval in Healthy Volunteers and Patients with Rheumatoid Arthritis or IgG4-Related Disease

    Claire Mukashyaka1, Xiaodong Wang1, Sujata Arora1, Mason Yamashita1, Allen Poma1 and Tanya Fischer2, 1Zenas BioPharma, Waltham, MA, 2Zenas BioPharma, Waltham, CA

    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 benefit…
  • Abstract Number: 2507 • ACR Convergence 2024

    IgG4-related Disease Features and Immunological Profile in a Tertiary Center in Sao Paulo-Brazil

    Frederico Pinheiro1, Cristina Orikaza2, Gerson Keppeke3, Emília Sato2 and Alexandre de Souza4, 1Universidade Federal de Sao Paulo - UNIFESP, São Paulo, SP, Brazil, 2Universidade Federal de Sao Paulo, São Paulo, SP, Brazil, 3Universidad Catolica del Norte, São Paulo, Coquimbo, Brazil, 4UNIFESP-EPM, São Paulo, SP, Brazil

    Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic, fibroinflammatory condition affecting almost all organs. It is a rare entity that is continuously being studied worldwide. It…
  • Abstract Number: 0736 • ACR Convergence 2024

    Treatment of IgG4-Related Disease (IgG4-RD) with Elotuzumab, an Inhibitor of SLAM-F7: Report of a Phase 2 Clinical Trial

    John Stone1, Arezou Khosroshahi2, Shounak Majumder3, Cory Perugino4, Ana Fernandes4, Grace McMahon4, Isha Jha4, John Varghese2, Adriana Delgado3, Guy Katz4, Zachary Wallace5, Shiv Pillai4, William Barry6, Ashley Pinckney7, Ellen Goldmuntz8, Beverly Welch9 and Kaitlin McShea9, 1Massachusetts General Hospital , Harvard Medical School, Concord, MA, 2Emory University, Atlanta, GA, 3Mayo Clinic, rochester, MN, 4Massachusetts General Hospital, Boston, MA, 5Massachusetts General Hospital, Newton, MA, 6Rho, Durham, NC, 7Rho, St Louis Park, NC, 8NIAID/ NIH, Washington, DC, 9Rho, Washington, DC

    Background/Purpose: CD4+ cytotoxic T lymphocytes (CD4+ CTLs), plasmablasts, and selected other cells of the T and B cell lineages have been identified as cellular linchpins of…
  • Abstract Number: 0753 • ACR Convergence 2024

    Treatment and Evolution in 134 Patients with Aortitis and Periaortitis. Experience of a Single Referral Centre

    Carmen Secada Gómez1, Javier Loricera2, Adrián Martín-Gutiérrez3, Fernando Lopez-Gutierrez4, Santos Castañeda5 and Ricardo Blanco-Alonso6, 1Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 2Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 3Hospital Universitario Marques de Valdecilla, IDIVAL, Renedo de Piélagos, Cantabria, Spain, 4Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Cantabria, Spain, 5Hospital Universitario de la Princesa, Madrid, Spain, 6Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain

    Background/Purpose: Aortitis and periaortitis include a heterogeneous group of entities that may be idiopathic or secondary to infectious and non-infectious processes. Therefore, treatment will differ…
  • Abstract Number: 0754 • ACR Convergence 2024

    Clinical Characterization of Aortitis and Periaortitis in a Cohort of 134 Patients from a Single Universitary Center. A Model-based Cluster Analysis

    Carmen Secada Gómez1, Javier Loricera2, Adrián Martín-Gutiérrez3, Fernando Lopez-Gutierrez4, María Nuñez-Sayar5, Ander Ucelay-Aristi5, Isabel Martinez-Rodriguez6, ivan Ferraz-Amaro7, Santos Castañeda8 and Ricardo Blanco-Alonso9, 1Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 2Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 3Hospital Universitario Marques de Valdecilla, IDIVAL, Renedo de Piélagos, Cantabria, Spain, 4Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Cantabria, Spain, 5Cardiovascular Surgery, Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain, 6Nuclear Medicine, Hospital Universitario Marques de Valdecilla,IDIVAL, Santander, Cantabria, Spain, 7Rheumatology, Hospital Universitario de Canarias, Santa Cruz de Tenerife, Canarias, Spain, 8Hospital Universitario de la Princesa, Madrid, Spain, 9Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain

    Background/Purpose: Aortitis and periaortitis represent the inflammation of the aortic wall and the surrounding periaortic tissues, respectively. Both can be idiopathic or secondary to infectious…
  • Abstract Number: 0775 • ACR Convergence 2024

    A Phase 3, Randomized, Double-Blind, Multicenter, Placebo-Controlled Study of Inebilizumab in IgG4-Related Disease (MITIGATE): Primary Efficacy and Safety Findings

    John Stone1, Emma Culver2, Arezou Khosroshahi3, Wen Zhang4, Emanuel Della Torre5, Kazuichi Okazaki6, Yoshiya Tanaka7, Matthias Löhr8, Nicolas Schleinitz9, Lingli Dong10, Hisanori Umehara11, Marco Lanzillotta12, Zachary Wallace13, Mikael Ebbo14, George Webster15, Ferran Martinez Valle16, Manu Nayar17, Vinciane Rebours18, Cory Perugino19, Xinxin Dong20, Yanping Wu20, Nishi Rampal20 and Daniel Cimbora20, 1Massachusetts General Hospital , Harvard Medical School, Concord, MA, 2John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom, 3Emory University, Atlanta, GA, 4Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), 5Ospedale San Raffaele, Milan, Italy, 6University of Occupational and Environmental Health, Osaka, Japan, 7Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Fukuoka, Japan, 8Karolinska Institutet, Stockholm, Sweden, 9Aix Marseille university, AP-HM, Marseille, France, 10Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China (People's Republic), 11Department of Rheumatology and Immunology, Nagahama City Hospital,, Nagahama, Shiga 526-0043, Japan, Japan, 12IRCCS Ospedale San Raffaele, Milano, Italy, 13Massachusetts General Hospital, Newton, MA, 14Aix-Marseille Université, Marseille, France, 15University College Hospital London, London, United Kingdom, 16Vall d’Hebron Hospital, Barcelona, Spain, 17Freeman Hospital, Newcastle Upon Tyne, United Kingdom, 18Beaujon Hospital, Université Paris-Cité, France, 19Massachusetts General Hospital, Boston, MA, 20Amgen Inc., Thousand Oaks, CA

    Background/Purpose: IgG4-related disease (IgG4-RD) is a rare, systemic, fibroinflammatory disease characterized by unpredictable and recurring flares, leading to organ damage and decreased quality of life.…
  • Abstract Number: 1113 • ACR Convergence 2024

    2nd Analysis of IgG4-Related Disease in a Diverse Long Island Population: Utilizing Serological and Histological Markers for Assessing Treatment Response

    Young Min Cho1, Alejandro Quintero Villegas2, Matt Means3, Zanos Theodoros2, Lara El Khoury4 and Galina Marder3, 1Northwell, Glen Cove, NY, 2Feinstein Institutes for Medical Research, Manhasset, NY, 3Northwell, Manhasset, NY, 4Division of Rheumatology/Northwell Health, Great Neck, NY

    Background/Purpose: IgG4-RD presents various clinical manifestations, making it challenging to assess treatment responses.  B-cell depletion agents such as Rituximab (RTX) are used in refractory cases.…
  • 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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