Abstracts tagged "IgG4 Related Disease"

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: 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: 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…
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…

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