Abstracts tagged "IgG4 Related Disease"

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: 0255 • ACR Convergence 2023
Lymphoid Mass in the Inferior Turbinate with IgG4 Producing Cells: A Nasal Manifestation of IgG4-RD or a Distinct Chronic Inflammatory Disease?
Ji In Jung¹, Ju Yeon Kim², Jun Won Park², Eun Bong Lee², Jin Kyun Park³ and Doo Hee Han², ¹Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, South Korea, ²Seoul National University Hospital, Seoul, South Korea, ³Seoul National University College of Medicine, Seoul, South Korea
Background/Purpose: IgG4-related disease (IgG4-RD) is a rare, multi-system, fibro-inflammatory disorder characterized by the infiltration of lymphoplasmacytic cells, storiform fibrosis, obliterative phlebitis, and the presence of…
Abstract Number: 1943 • ACR Convergence 2023
IgG4-related Disease: 2010-2022 Case Review and Comparative Evaluation of Diagnostic Criteria
Pablo Martínez Calabuig¹, Jorge Juan Fragio¹, Roxana Gonzalez Mazarío², Amalia Rueda Cid³, Mireia Lucía Sanmartín Martínez¹, Laura Salvador Maicas¹, Laura Abenza Barberá¹, María Cristina Sabater Abad¹, Antonio Sierra Rivera¹, Inmaculada Castelló Miralles¹, Juan José Lerma Garrido¹, Clara Molina Almela³, Isabel Balaguer Trull¹ and Cristina Campos Fernández¹, ¹Hospital General Universitario Valencia, Valencia, Spain, ²Hospital de Sagunto, Valencia, Spain, ³Hospital General de Valencia, Valencia, Spain
Background/Purpose: IgG4 immunoglobulin-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory process with an unclear etiology and pathophysiology with the capacity of affecting multiple organs.…
Abstract Number: 0257 • ACR Convergence 2023
Activity of IgG4-related Disease Differs Depending on the Presence or Absence of Concomitant Hypocomplementemia
Risa Wakiya¹, Hiroki Ozaki², Hiromi Shimada¹, Shusaku Nakashima¹, Taichi Miyagi³, Yusuke Ushio⁴, Koichi Sugihara⁵, Mao Mizusaki¹, Rina Mino⁶, Kanako Chujo⁷, Ryoko Kagawa⁴, Hayamasa Yamaguchi⁴, Tomohiro Kameda¹ and Hiroaki Dobashi¹, ¹Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kagawa, Japan, ²Department of Rheumatology, KKR Takamatsu Hospital, Kagawa, Japan, ³Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kidagun, Japan, ⁴Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Miki-cho, Kita-gun, Japan, ⁵Kagawa University, Miki-cho, Kita-gun, Japan, ⁶Kagawa University, Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa, Japan, ⁷Kagawa University, Miki, Kita District, Kagawa, Japan
Background/Purpose: Hypocomplementemia (HC) is often observed in IgG4-related diseases (IgG4-RD), but there are also IgG4-RD without HC. This study aimed to clarify the difference of…
Abstract Number: 2001 • ACR Convergence 2023
Burden of Metabolic Bone Disease in Patients with IgG4-Related Disease with and Without Autoimmune Pancreatitis
Guy Katz¹, Aubree McMahon¹, Grace McMahon¹, Isha Jha¹, Marcy Bolster², Ana Fernandes¹, Zachary Wallace³, Cory Perugino¹, John Stone⁴ and Yasmin Hernandez-Barco¹, ¹Massachusetts General Hospital, Boston, MA, ²Massachusetts General Hospital, Concord, MA, ³Massachusetts General Hospital, Newton, MA, ⁴Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Concord, MA
Background/Purpose: Metabolic bone disease (MBD), including osteopenia and osteoporosis, is common in patients with inflammatory disorders, due to disease factors and glucocorticoid (GC) use, and…
Abstract Number: 0271 • ACR Convergence 2023
Successful Treatment of Refractory IgG4-Related Disease with Tofacitinib: Experiences from 7 Patients
Huaqun Zhu, Sumei Tang, Yun Li, Feng Sun, Yuzhou Gan and Hua Ye, Peking University People’s Hospital, Beijing, China
Background/Purpose: Our observational cohort study aimed at assessing the effectiveness and safety of the Janus kinase (JAK) inhibitor tofacitinib in refractory IgG4-Related Disease (IgG4-RD).Methods: Seven…
Abstract Number: 2089 • ACR Convergence 2023
Social Listening Analysis of IgG4-Related Disease Social Media Discussions
Esteban Rivera¹, Kelly Gavigan², Daniel Hernandez³, Maria Picone⁴, Angeni Cordova⁴, Lauren Dougherty⁴, Kristina Davidson⁵, Brian LaMoreaux⁶, Anthony Amatucci⁷ and W. Benjamin Nowell⁸, ¹Global Healthy Living Foundation, Long Island City, NY, ²Global Healthy Living Foundation, Upper Nyack, NY, ³Global Healthy Living Foundation, St. Johns, FL, ⁴TREND Community, Philadelphia, PA, ⁵Horizon Therapeutics, Dublin, Ireland, ⁶Horizon Therapeutics, Deerfield, IL, ⁷Horizon Therapeutics, Cambridge, MA, ⁸Global Healthy Living Foundation, Nyack, NY
Background/Purpose: Immunogobulin G4-Related Disease (IgG4-RD) is a rare chronic immune-mediated fibroinflammatory disorder that often manifests with tumor-like masses and/or enlargement of multiple organs. In order…
Abstract Number: 0273 • ACR Convergence 2023
Disease Characteristics and Pancreatic Damage in IgG4-Related Disease with Pancreatic Involvement
Guy Katz¹, Liam Harvey², Yasmin Hernandez-Barco¹, Ana Fernandes¹, Grace McMahon¹, Isha Jha¹, Zachary Wallace³, Cory Perugino¹ and John Stone⁴, ¹Massachusetts General Hospital, Boston, MA, ²Frank H. Netter School of Medicine at Quinnipiac University, New Haven, CT, ³Massachusetts General Hospital, Newton, MA, ⁴Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Concord, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory disease that can affect nearly every organ system. Autoimmune pancreatitis (AIP) is among the most common…
Abstract Number: 0330 • ACR Convergence 2023
Patient-Reported Experiences and Comorbidities in Patients with IgG4-Related Disease with and Without Pancreatic Involvement
Liam Harvey¹, Guy Katz², Yasmin Hernandez-Barco², Ana Fernandes², Aubree McMahon², Grace McMahon², Isha Jha², Zachary Wallace³, Cory Perugino² and John Stone⁴, ¹Frank H. Netter School of Medicine at Quinnipiac University, New Haven, CT, ²Massachusetts General Hospital, Boston, MA, ³Massachusetts General Hospital, Newton, MA, ⁴Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Concord, MA
Background/Purpose: IgG4-Related Disease (IgG4-RD) is a systemic immune-mediated disease that commonly involves the pancreas in the form of autoimmune pancreatitis (AIP). Despite being one of…
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…

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