IgG4 Related Disease Archives - ACR Meeting Abstracts

Abstract Number: 0676 • ACR Convergence 2026
Clinical Phenotype of Overlapping IgG4-Related Disease and ANCA-Associated Vasculitis: A Multicenter Case Series
marie Nansion¹, Matthieu Groh², Belfeki Nabil³, Carole Lacout⁴, Anouk Legoueff⁵, Sophie Gallou⁶, Achille Aouba⁷, Rafik MESBAH⁸, Cedric Rafat⁹, Sara Melboucy¹⁰, Jean SIBILIA¹¹, Benjamin Terrier¹² and Marc Scherlinger¹³ Affiliations: ¹Strasbourg University Hospital, Strasbourg, Alsace, France, ²Internal Medicine Department, Clinical Immunology and Hematology, Foch Hospital, National Reference Center for Hypereosinophilic Syndromes, Versailles Saint Quentin-en-Yvelines University, Surenes, France, ³Department of Internal Medicine, Melun Hospital, Melun, France, Melun, France, ⁴Internal Medicine Department, Angers Hospital, Angers, France, ⁵Luxembourg, Luxembourg, Luxembourg, ⁶Hopitaux Universitaires de Caen, Caen, France, ⁷Caen University Hospital, Caen, ⁸Department of Nephrology, Boulogne-sur-Mer General Hospital, Boulogne-sur-mer, France, ⁹APHP, Paris, France, ¹⁰Department of Internal Medicine, Saint-Quentin General Hospital, Saint-Quentin, France, ¹¹Hôpitaux Universitaires de Strasbourg, STRASBOURG, Alsace, France, ¹²Internal Medicine Department, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, Cochin Hospital, APHP, Paris Cité University, Paris, France, ¹³Strasbourg University Hospital, Strasbourg, France
Background/Purpose: IgG4-related disease (IgG4-RD) and ANCA-associated vasculitis (AAV) can share organ involvement, inflammatory histology, and serologic abnormalities, creating diagnostic uncertainty. We described clinical, serologic, histopathologic,…
Abstract Number: 2107 • ACR Convergence 2026
Efficacy and Safety of Inebilizumab for the Treatment of IgG4-RD Across Age Subgroups: Analysis from the Open-label Period of the MITIGATE Trial
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, Mikael Ebbo¹³, Fernando Martínez-Valle¹⁴, Manu Nayar¹⁵, Vinciane Rebours¹⁶, Cory Perugino¹⁷, Sarah Bray¹⁸, Zachary Wallace¹⁹, Kelly Steed²⁰, Maya Osman²¹, Sue Cheng²⁰ and Daniel Cimbora²⁰ Affiliations: ¹Division of Rheumatology, Inflammation, and Immunity, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA, Concord, MA, ²Translational Gastroenterology and Liver Unit, John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, oxford, England, United Kingdom, ³Division of Rheumatology, Emory University School of Medicine, Atlanta, GA, ⁴Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), ⁵Università Vita-Salute San Raffaele, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UNIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy, Milan, Italy, ⁶Department of Internal Medicine, Kansai Medical University Kori Hospital, Osaka, Japan, ⁷Department of Molecular Targeted Therapeutics, University of Occupational and Environmental Health, Kitakyushu, Japan, ⁸Department of Clinical Science, Intervention, and Technology, Karolinska Institutet, Stockholm, Sweden, ⁹Departement de Médecine Interne, Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France, ¹⁰Huazhong University of Science and Technology, Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Wuhan, China (People's Republic), ¹¹Department of Rheumatology and Immunology, Nagahama City Hospital, Nagahama, Shiga 526-0043, Japan, Japan, ¹²IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy, and Rare Diseases, Milan, Italy, ¹³Departement de Medecine Interne Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France, ¹⁴Hospital Universitari Vall d'Hebron, Barcelona, Spain, Barcelona, Spain, ¹⁵Freeman Hospital, Hepato-Pancreato-Biliary Unit, Newcastle upon Tyne, United Kingdom, ¹⁶Université Paris-Cité, Pancreatology and Digestive Oncology Department, Beaujon Hospital, AP-HP, Clichy, Paris, France, ¹⁷Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ¹⁸Amgen Ltd., Cambridge, United Kingdom, ¹⁹Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, and Harvard Medical School, Boston, MA, ²⁰Amgen Inc., Thousand Oaks, CA, ²¹Amgen Inc., Dearborn, MI
Background/Purpose: Immunoglobulin G4-related disease (IgG4-RD) is a chronic, systemic fibroinflammatory disease characterized by unpredictable and recurrent flares, leading to progressive organ damage and decreased quality…
Abstract Number: 0678 • ACR Convergence 2026
Endothelial Cells-Mediated Immune Cell Infiltration Promotes Retroperitoneal Fibrosis: Interrogation of Mechanisms and Translational Potential
Gao Hui¹, Yuanbang Mai², Shibo Liu³, Yanwen Wang⁴, Zhanguo Li⁵, Chenghua Luo⁶, Hengjia Liu⁴ and Cuiping Pan² Affiliations: ¹Tsinghua University Beijing Tsinghua Changgung Hospital, Beijing, China (People's Republic), ²The Hong Kong University of Science and Technology (Guangzhou), Guangzhou, China (People's Republic), ³Peking University International Hospital, Beijing, China (People's Republic), ⁴Zhejiang University, Haining, China (People's Republic), ⁵Peking univeristy people's hospital, Beijing, Beijing, China, ⁶Peking University People's Hospital, Beijing, China (People's Republic)
Background/Purpose: Idiopathic retroperitoneal fibrosis (RPF) is a rare autoimmune disease characterized by inflammation and fibrotic tissue growth in the retroperitoneum. Its etiopathogenesis remains unclear.Methods: Single-cell…
Abstract Number: 2108 • ACR Convergence 2026
Epidemiology and Outcomes of IgG4-Related Disease in a Nationwide United States Cohort
Remco Kersten¹, Osamu Yasui², Esther Velasquez³, Sachi Bopardikar⁴, Neil Kamdar³ and Matthew Baker⁵ Affiliations: ¹Department of Medicine, Stanford University, Palo Alto, CA, ²Division of Cardiothoracic Anesthesiology, Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University, Palo Alto, CA, ³Center for Population Health Sciences, Stanford University, Palo Alto, ⁴Division of Immunology and Rheumatology, Department of Medicine, Stanford University,, Palo Alto, ⁵Division of Immunology and Rheumatology, Department of Medicine, Stanford University, Stanford, CA, USA, San Carlos, CA
Background/Purpose: To establish and characterize a nationwide real-world cohort of IgG4-related disease (IgG4-RD) using the recently introduced ICD-10-CM code and evaluate disease epidemiology, organ phenotypes,…
Abstract Number: 0689 • ACR Convergence 2026
Organ Damage in IgG4-Related Disease is Common at the Time of Initial Evaluation and is Driven by Disease Duration
Guy Katz¹, Federica Bonaso², Ana Fernandes³, Manaswini Pujar¹, Nora Shepherd⁴, Katherine Davis¹, Andrea Meyers⁵, Rajvi Wani⁵, Melissa Rosen⁶, Kelly Steed⁶, Cory Perugino⁷ and John Stone⁸ Affiliations: ¹Massachusetts General Hospital, Boston, MA, ²University of Brescia/MGH, Monza, Monza and Brianza, Italy, ³Medical School Candidate, Boston, MA, ⁴George Washington University, Boston, MA, ⁵Amgen, Inc., Thousand Oaks, CA, ⁶Amgen Inc., Thousand Oaks, CA, ⁷Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ⁸Division of Rheumatology, Inflammation, and Immunity, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA, Concord, MA
Background/Purpose: IgG4-related disease (IgG4-RD) can expose patients to a variety of complications, including organ dysfunction from destructive inflammatory infiltrate or fibrosis and diagnostic or therapeutic…
Abstract Number: 2109 • ACR Convergence 2026
Immunogenicity and Impact of Anti-Drug Antibodies on the Efficacy and Pharmacokinetics of Inebilizumab in a Phase 3 Trial in IgG4-Related Disease
Arezou Khosroshahi¹, Melissa Rosen², John Stone³, Emma Culver⁴, Wen Zhang⁵, Emanuel Della-Torre⁶, Kazuichi Okazaki⁷, Yoshiya Tanaka⁸, Matthias Löhr⁹, Nicolas Schleinitz¹⁰, Lingli Dong¹¹, Hisanori Umehara¹², Marco Lanzillotta¹³, Zachary Wallace¹⁴, Mikael Ebbo¹⁵, George Webster¹⁶, Fernando Martínez-Valle¹⁷, Manu Nayar¹⁸, Vinciane Rebours¹⁹, Cory Perugino²⁰, Cody Peer², Rong Luo², Sue Cheng² and Daniel Cimbora² Affiliations: ¹Division of Rheumatology, Emory University School of Medicine, Atlanta, GA, ²Amgen Inc., Thousand Oaks, CA, ³Division of Rheumatology, Inflammation, and Immunity, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA, Concord, MA, ⁴Translational Gastroenterology and Liver Unit, John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, oxford, England, United Kingdom, ⁵Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), ⁶Università Vita-Salute San Raffaele, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UNIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy, Milan, Italy, ⁷Department of Internal Medicine, Kansai Medical University Kori Hospital, Osaka, Japan, ⁸Department of Molecular Targeted Therapeutics, University of Occupational and Environmental Health, Kitakyushu, Japan, ⁹Department of Clinical Science, Intervention, and Technology, Karolinska Institutet, Stockholm, Sweden, ¹⁰Departement de Médecine Interne, Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France, ¹¹Huazhong University of Science and Technology, Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Wuhan, China (People's Republic), ¹²Department of Rheumatology and Immunology, Nagahama City Hospital, Nagahama, Shiga 526-0043, Japan, Japan, ¹³IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy, and Rare Diseases, Milan, Italy, ¹⁴Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, and Harvard Medical School, Boston, MA, ¹⁵Departement de Medecine Interne Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France, ¹⁶Pancreaticobiliary Medicine, University College London Hospitals, London, United Kingdom, ¹⁷Hospital Universitari Vall d'Hebron, Barcelona, Spain, Barcelona, Spain, ¹⁸Freeman Hospital, Hepato-Pancreato-Biliary Unit, Newcastle upon Tyne, United Kingdom, ¹⁹Université Paris-Cité, Pancreatology and Digestive Oncology Department, Beaujon Hospital, AP-HP, Clichy, Paris, France, ²⁰Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA
Background/Purpose: Immunoglobulin G4-related disease (IgG4-RD) is a progressive, systemic, fibroinflammatory disease characterized by unpredictable and recurring flares, leading to organ damage and decreased quality of…
Abstract Number: 0690 • ACR Convergence 2026
Pancreatic and Extrapancreatic IgG4-Related Disease Relapses are Common Following Treatment Initiation Across All Autoimmune Pancreatitis Phenotypes
Nora Shepherd¹, Manaswini Pujar², Katherine Davis², Ana Fernandes³, Cory Perugino⁴, John Stone⁵, Yasmin Hernandez-Barco² and Guy Katz² Affiliations: ¹George Washington University, Boston, MA, ²Massachusetts General Hospital, Boston, MA, ³Medical School Candidate, Boston, MA, ⁴Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ⁵Division of Rheumatology, Inflammation, and Immunity, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA, Concord, MA
Background/Purpose: Autoimmune pancreatitis (AIP) is one of the most common manifestations of IgG4-related disease (IgG4-RD), occurring in nearly 50% of patients. Several radiologic patterns of…
Abstract Number: 2110 • ACR Convergence 2026
Impact of Smoking on Clinical Outcomes in IgG4-Related Disease: A Propensity Score-Matched Analysis of a Large Federated Health Network
Arezou Khosroshahi¹ and Yurilu Gonzalez Moret² Affiliations: ¹Division of Rheumatology, Emory University School of Medicine, Atlanta, GA, ²Emory University, Atlanta, GA
Background/Purpose: Smoking is a modifiable risk factor with systemic pro-inflammatory effects, yet its impact on IgG4-related disease (IgG4-RD) outcomes has not been examined at scale.…
Abstract Number: 0696 • ACR Convergence 2026
Riedel’s thyroiditis, the major manifestation of IgG4-related disease in the thyroid gland: A series of 16 patients
Grace McMahon¹, Manaswini Pujar², Katherine Davis², Isha Jha³, Ana Fernandes⁴, Cory Perugino⁵, Guy Katz² and John Stone⁶ Affiliations: ¹Massachusetts General Hospital, Boston, ²Massachusetts General Hospital, Boston, MA, ³UMass Chan Medical School, Worcester, MA, ⁴Medical School Candidate, Boston, MA, ⁵Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ⁶Division of Rheumatology, Inflammation, and Immunity, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA, Concord, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is an immune-mediated disorder often characterized by high serum IgG4 concentrations, fibroinflammatory masses, and severe organ damage. Treatment responses across different…
Abstract Number: 2112 • ACR Convergence 2026
Long-Term Glucocorticoid Use and Clinical Outcomes in IgG4-Related Disease: A Nationwide Retrospective Cohort Study
Arezou Khosroshahi¹ and Yurilu Gonzalez Moret² Affiliations: ¹Division of Rheumatology, Emory University School of Medicine, Atlanta, GA, ²Emory University, Atlanta, GA
Background/Purpose: Systemic glucocorticoids remain the cornerstone of IgG4-related disease (IgG4-RD) treatment, yet their long-term safety profile in this population has not been characterized at scale.…
Abstract Number: 0698 • ACR Convergence 2026
Risk of Non-Hodgkin Lymphoma and All-Cause Mortality in IgG4-Related Disease Versus Sjögren Disease: A Retrospective Cohort Study
Anna Maria¹, Fabian Rodriguez Quinonez² and Irene Tan³ Affiliations: ¹St. Mary Medical Center, Langhorne, PA, ²Jefferson Einstein Hospital Philadelphia, Philadelphia, PA, ³Einstein Healthcare Network Philadelphia - Jefferson Health, Bala Cynwyd, PA
Background/Purpose: IgG4-related disease (IgG4-RD) and Sjögren disease (SjD) are chronic immune-mediated conditions reported to be associated with non-Hodgkin lymphoma (NHL). However, comparative real-world data evaluating…
Abstract Number: 2113 • ACR Convergence 2026
Long-Term Inebilizumab Treatment Results in Mild Immunoglobulin Reduction but No Increase in Infection Risk
Emanuel Della-Torre¹, John Stone², Emma Culver³, Arezou Khosroshahi⁴, Wen Zhang⁵, Kazuichi Okazaki⁶, Yoshiya Tanaka⁷, Matthias Löhr⁸, Nicolas Schleinitz⁹, Lingli Dong¹⁰, Hisanori Umehara¹¹, Marco Lanzillotta¹², Zachary Wallace¹³, Mikael Ebbo¹⁴, George Webster¹⁵, Fernando Martínez-Valle¹⁶, Manu Nayar¹⁷, Vinciane Rebours¹⁸, Cory Perugino¹⁹, Rong Luo²⁰, Maya Osman²¹, Sue Cheng²⁰ and Daniel Cimbora²⁰ Affiliations: ¹Università Vita-Salute San Raffaele, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UNIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy, Milan, Italy, ²Division of Rheumatology, Inflammation, and Immunity, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA, Concord, MA, ³Translational Gastroenterology and Liver Unit, John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, oxford, England, United Kingdom, ⁴Division of Rheumatology, Emory University School of Medicine, Atlanta, GA, ⁵Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), ⁶Department of Internal Medicine, Kansai Medical University Kori Hospital, Osaka, Japan, ⁷Department of Molecular Targeted Therapeutics, University of Occupational and Environmental Health, Kitakyushu, Japan, ⁸Department of Clinical Science, Intervention, and Technology, Karolinska Institutet, Stockholm, Sweden, ⁹Departement de Médecine Interne, Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France, ¹⁰Huazhong University of Science and Technology, Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Wuhan, China (People's Republic), ¹¹Department of Rheumatology and Immunology, Nagahama City Hospital, Nagahama, Shiga 526-0043, Japan, Japan, ¹²IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy, and Rare Diseases, Milan, Italy, ¹³Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, and Harvard Medical School, Boston, MA, ¹⁴Departement de Medecine Interne Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France, ¹⁵Pancreaticobiliary Medicine, University College London Hospitals, London, United Kingdom, ¹⁶Hospital Universitari Vall d'Hebron, Barcelona, Spain, Barcelona, Spain, ¹⁷Freeman Hospital, Hepato-Pancreato-Biliary Unit, Newcastle upon Tyne, United Kingdom, ¹⁸Université Paris-Cité, Pancreatology and Digestive Oncology Department, Beaujon Hospital, AP-HP, Clichy, Paris, France, ¹⁹Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ²⁰Amgen Inc., Thousand Oaks, CA, ²¹Amgen Inc., Dearborn, MI
Background/Purpose: Inebilizumab (INEB), a CD19-targeted B-cell depleting therapy, has demonstrated efficacy and safety in the treatment of immunoglobulin G4-related disease (IgG4-RD), as shown in the registrational…
Abstract Number: 0748 • ACR Convergence 2026
A Multimodal Optimal Biomarker Approach to Characterize Multicenter Patients with Retroperitoneal Fibrosis (RPF)
Liu Shibo¹, Cui Yang², Yubing Tong³ and Gao Hui⁴ Affiliations: ¹Peking University International Hospital, Beijing, China (People's Republic), ²University Medicine Mannheim, Mannheim, Germany, ³University of Pennsylvania, Philadelphia, ⁴Tsinghua University Beijing Tsinghua Changgung Hospital, Beijing, China (People's Republic)
Background/Purpose: Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disease that can mimic malignant retroperitoneal masses and many other retroperitoneal diseases. Accurate differentiation is essential, as treatment…
Abstract Number: 2120 • ACR Convergence 2026
Real-World Clinical Burden and Longitudinal Outcomes of IgG4-Related Disease: A Large-Scale Federated Health Network Analysis
Arezou Khosroshahi¹ and Yurilu Gonzalez Moret² Affiliations: ¹Division of Rheumatology, Emory University School of Medicine, Atlanta, GA, ²Emory University, Atlanta, GA
Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with poorly characterized real-world burden. We aimed to describe the clinical profile and longitudinal outcomes of…
Abstract Number: 0801 • ACR Convergence 2026
A New ICD-10 Code Opens a Real-World Window on IgG4-Related Disease: Propensity-Matched Outcomes of Rituximab Versus Conventional Immunosuppression, With an Early Look at Inebilizumab
SHILPA RAMAN DANAGARA¹, SALWA GHARIB², SATHWIK BILLA³, KRIMA PATEL⁴, PAVANA LALITHYA SAKHAMURI⁵ and SARWAT UMER⁶ Affiliations: ¹LSU Health Shreveport, shreveport, LA, ²Kansas University Medical Center Olathe, Kansas city, KS, ³Louisiana State University Health Sciences Center at Ochsner University Hospital & Clinics, Lafayette, LA, ⁴Baptist Hospitals of Southeast Texas, Beaumont, TX, ⁵Ochsner Lafayette General Hospital, Lafayette, LA, ⁶Center of Excellence for Arthritis and Rheumatology, LSU Health, Shreveport, LA
Background/Purpose: IgG4-related disease (IgG4-RD) is a rare, multi-organ fibroinflammatory condition with scarce comparative real-world treatment data, and until October 2023 it lacked a dedicated diagnostic…

Abstracts tagged "IgG4 Related Disease"