Abstracts tagged "IgG4 Related Disease"

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…
Abstract Number: 2027 • ACR Convergence 2024
Clinical Features and Treatment of Retroperitoneal Fibrosis in a Referral Hospital and Literature Review
Adrián Martín-Gutiérrez¹, Javier Loricera², Fernando Lopez-Gutierrez³ and Ricardo Blanco-Alonso⁴, ¹Hospital Universitario Marques de Valdecilla, IDIVAL, Renedo de Piélagos, Cantabria, Spain, ²Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ³Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Cantabria, Spain, ⁴Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain
Background/Purpose: Retroperitoneal fibrosis (RPF) is usually idiopathic (iRPF), but may be secondary to drugs, neoplasms, infections, or may be part of an IgG4-related disease. Our…
Abstract Number: 2031 • ACR Convergence 2024
A Contemporary Assessment of the Burden of Malignancy in IgG4-Related Disease
Grace McMahon¹, Isha Jha¹, Guy Katz¹, Cory Perugino¹, Ana Fernandes¹, 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: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder that can affect nearly any organ. Because of the often tumefactive nature of IgG4-RD and its typical…
Abstract Number: 2035 • ACR Convergence 2024
Older Age and Higher Erythrocyte Sedimentation Rate Are Associated with Increased Disease Burden at Diagnosis in a Cohort of IgG4-Related Disease Patients
Humberto Campos Clemente, Barbara Bayeh, Monica Raquel de Souza Aquino, Fernanda Oliveira de Andrade Lopes, Thiago Quadrante Freitas, Rafael Alves Cordeiro and Henrique Ayres Mayrinki Giardini, Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil, São Paulo, SP, Brazil
Background/Purpose: Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition primarily affecting middle-aged males. It is characterized by mass lesions affecting various organs, such as the…
Abstract Number: 0007 • ACR Convergence 2024
Bromodomain 2 of BET Proteins Plays a Crucial Role in Follicular/peripheral Helper T Cell and Plasma Cell Differentiation
Ryota Sato¹, Mitsuhiro Akiyama², Koichi Oshita³, Yuko Kawano³, Yuki Imura³, Piruzyan Mariam⁴, Manami Kikuchi³, Mikako Murase³, Sachiko Mochiduki³, Naohiro Hashimoto³, Masayuki Watanabe³, Keiko Yoshimoto⁵, Tsutomu Takeuchi⁶, Kenji Chiba⁷ and Yuko Kaneko⁵, ¹Mitsubishi Tanabe Pharma Corporation, Brighton, MA, ²Keio University School of Medicine, Palo Alto, CA, ³Mitsubishi Tanabe Pharma Corporation, Yokohama, Japan, ⁴Mitsubishi Tanabe Pharma Corporation, Yokohama, Kanagawa, Japan, ⁵Keio University School of Medicine, Tokyo, Japan, ⁶Department of Internal Medicine, Keio University, Tokyo, Tokyo, Japan, ⁷Mitsubishi Tanabe Pharma Corporation, Tokyo, Japan
Background/Purpose: Proteins motifs bromodomain 1 (BD1) and BD2 within the BET family proteins regulate gene expression of inflammatory cytokines and lymphocyte proliferation by binding to…
Abstract Number: 2037 • ACR Convergence 2024
Can Tofacitinib Be the Saviour in Treatment of Refractory IgG4-Related Disease : A Multicentre Experience from India
Jasneet Singh¹, SHRUTI BAJAD² and DHAVAL TANNA³, ¹THE MEDICLINIC-ARTHRITIS & RHEUMATOLOGY CENTER, LUDHIANA, Punjab, India, ²Moolchand hospital , Delhi, DELHI, Delhi, India, ³SAURASHTRA RHEUMATOLOGY CENTER, Rajkot, Gujarat, India
Background/Purpose: We did an observational study to assess the efficacy and safety of the Janus kinase (JAK) inhibitor tofacitinib in patients having refractory 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…

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