Abstracts tagged "IgG4 Related Disease"

Abstract Number: 1785 • 2018 ACR/ARHP Annual Meeting
A Data-Driven Approach to Guide Physicians When Considering the Differential Diagnosis of IgG4-Related Disease
Mark A. Matza¹, Zachary Wallace² and John H. Stone³, ¹Rheumatology Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ²Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ³Rheumatology (Medicine), Massachusetts General Hospital, Harvard Medical School, Boston, MA
Background/Purpose: IgG4-Related Disease (IgG4-RD) is a chronic immune-mediated fibro-inflammatory disorder of unknown etiology. The differential diagnosis is broad, partly because of the myriad potential organ…
Abstract Number: 1787 • 2018 ACR/ARHP Annual Meeting
How to Better Diagnose IgG4 Related Disease: a Single-Center Based Experience
Anji Xiong¹, Yuan Yang¹, Beibei Cui¹, Jianhong Sun¹, Qibing Xie¹, Yi Zhao¹, Chunyu Tan¹, Min Yang¹, Yi Liu¹, Honghu Tang¹, Pingying Qing¹, Lingshu Zhang¹, Yubin Luo¹, Yan Liang¹, Ying Wang¹, Yali Ye¹, Ling Ma¹, Shiyu Yi¹ and Yi Liu², ¹Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China, ²Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, Sichuan, China, Chengdu, China
Background/Purpose: Pathology associated with IgG4-related disease (IgG4-RD) has been described in virtually every tissue and organ of the body. The heterogeneous clinical manifestations of IgG4-RD…
Abstract Number: 138 • 2018 ACR/ARHP Annual Meeting
Distinct Roles of Tfh2, SLAMF7+ Tfh1 Cells and Th1 Cells in the Pathogenesis of IgG4-RD
Kazuhiko Higashioka¹, Masahiro Ayano¹, Yasutaka Kimoto², Hiroki Mitoma¹, Mitsuteru Akahoshi¹, Yojiro Arinobu¹, Koichi Akashi¹, Takahiko Horiuchi³ and Hiroaki Niro⁴, ¹Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan, ²Department of Internal Medicine, Kyushu University Beppu Hospital, Oita, Japan, ³Department of Internal Medicine, Kyushu University Beppu Hospital, Beppu, Japan, ⁴Department of Medical Education, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan
Background/Purpose: IgG4-related disease (IgG4-RD) is a novel disease entity characterized by the infiltration of IgG4-secreting plasmablasts (PBs) and the generation of germinal centers in various…
Abstract Number: 370 • 2018 ACR/ARHP Annual Meeting
Rituximab in Idiopathic Retroperitoneal Fibrosis
Veronika Boyeva¹, Hatim Alabsi², Michael Seidman³, Ryan Paterson⁴, Jason Kur⁵, Silvia Chang⁶, Luke Chen⁷ and Mollie Carruthers⁵, ¹Department of Medicine, University of British Columbia, Vancouver, BC, Canada, ²Department of Radiology, King Abdulaziz University, Jeddah, Saudi Arabia, ³Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada, ⁴Department of Urology, University of British Columbia, Vancouver, BC, Canada, ⁵Rheumatology, University of British Columbia, Vancouver, BC, Canada, ⁶Department of Radiology, University of British Columbia, Vancouver, BC, Canada, ⁷Department of Hematology, University of British Columbia, Vancouver, BC, Canada
Background/Purpose: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the proliferation of fibrous tissue in the retroperitoneum, most commonly surrounding the aorta from the…
Abstract Number: 371 • 2018 ACR/ARHP Annual Meeting
Therapeutic Efficacy and Safety of Iguratimod in Treating Mild IgG4-Related Diseases
Wen Zhang¹ and Panpan Zhang², ¹Rheuamtology, Peking Union Medical College Hospital, Beijing, China, ²Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China
Background/Purpose: To evaluate the therapeutic efficacy and safety of Iguratimod in the treatment of mild IgG4 related disease (IgG4-RD). Methods: Thirty patients with newly diagnosed…
Abstract Number: 1779 • 2018 ACR/ARHP Annual Meeting
Is the Number of IgG4+ Plasma Cells Observed By Immunostaining Important Beyond Its Diagnostic Utility in IgG4-Related Disease?
Eduardo Martín Nares¹, Jacobo Guerrero Castillo², Arturo Angeles Angeles² and Gabriela Hernandez-Molina¹, 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: The histopathological findings in IgG4-related disease (IgG4-RD) includes the presence of dense lymphoplasmacytic infiltrate, obliterative phlebitis, storiform fibrosis and the presence of marked IgG4+…
Abstract Number: 1781 • 2018 ACR/ARHP Annual Meeting
Retrospective Analysis of IgG4-RD Patient Population at the Cleveland Clinic between 2007-2017
Chan Mi Lee¹, Mohamed Alalwani², Richard Prayson^1,3 and Carmen E. Gota^1,4, ¹Education, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, ²Hospital Medicine, Cleveland Clinic, Cleveland, OH, ³Anatomic Pathology, Cleveland Clinic, Cleveland, OH, ⁴Orthopedic and Rheumatologic Institute, Cleveland Clinic, Cleveland, OH
Background/Purpose: IgG4-related disease (IgG4-RD) is a rare multisystem fibro-inflammatory condition, characterized by organ mass lesions, IgG4+ lymphoplasmacytic infiltrate, and storiform fibrosis. To gain better understanding…
Abstract Number: 1783 • 2018 ACR/ARHP Annual Meeting
Salivary Gland Disease in IgG4-Related Disease Is Associated with Allergic Histories
Samantha Sanders¹, Emanuel Della Torre², Cory A. Perugino³, Aidan Long⁴, Hyon K. Choi⁴, John H. Stone⁵ and Zachary Wallace⁶, ¹Harvard Medical School, Boston, MA, ²Unit of Medicine and Clinical Immunology, San Raffaele Scientific Institute, Milan, Italy, ³Ragon Institute of MGH, MIT and Harvard, Cambridge, MA, ⁴Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston, MA, ⁵Rheumatology (Medicine), Massachusetts General Hospital, Harvard Medical School, Boston, MA, ⁶Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA
Background/Purpose: The etiology of IgG4-related disease (IgG4-RD) remains unknown. The role of T-helper type 2 (Th2) cells in the pathogenesis of IgG4-RD is controversial. Given…
Abstract Number: 4L • 2017 ACR/ARHP Annual Meeting
Final Results of an Open Label Phase 2 Study of a Reversible B Cell Inhibitor, Xmab®5871, in IgG4-Related Disease
John H. Stone¹, Zachary S. Wallace², Cory A. Perugino³, Ana D. Fernandes⁴, Payal Patel⁵, Paul A. Foster⁶ and Debra J. Zack⁶, ¹Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, ²Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ³Ragon Institute of MGH, MIT and Harvard, Cambridge, MA, ⁴Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston, MA, ⁵Rheumatology, Harvard, Boston, MA, ⁶Xencor, Inc., San Diego, CA

Background/Purpose: IgG4-related disease (IgG4-RD) is an immune-mediated condition causing fibro-inflammatory lesions that can lead to irreversible organ damage and death. No approved therapies exist. A…
Abstract Number: 1175 • 2017 ACR/ARHP Annual Meeting
Rituximab for Idiopathic and IgG4-Related Retroperitoneal Fibrosis
Rachel Wallwork¹, Zachary S. Wallace², Cory A. Perugino³, Amita Sharma⁴ and John H. Stone⁵, ¹Department of Medicine, Massachusetts General Hospital, Boston, MA, ²Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ³Ragon Institute of MGH, MIT and Harvard, Cambridge, MA, ⁴Department of Radiology, Massachusetts General Hospital, Boston, MA, ⁵Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA
Rituximab for Idiopathic and IgG4-Related Retroperitoneal FibrosisBackground/Purpose: Untreated retroperitoneal fibrosis (RPF) can lead to chronic back and flank pain and/or renal failure. The mainstay of…
Abstract Number: 1180 • 2017 ACR/ARHP Annual Meeting
Multicenter Study on the Rate of Renal Function Deterioration in IgG4-Related Tubulointerstitial Nephritis
Mitsuhiro Kawano¹, Ichiro Mizushima², Takahiro Matsunaga¹, Kazunori Yamada³, Satoshi Hara¹, Hiroshi Fujii¹, Takako Saeki⁴, Yoshinori Taniguchi⁵ and Hitoshi Nakashima⁶, ¹Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan, ²Kanazawa University Hospital, Kanazawa, Japan, ³Department of Advanced Research in Community Medicine, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan, ⁴Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan, ⁵Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi Medical School, Kochi, Japan, ⁶Div of Nephrol & Rheumatol, Dept of Int Med, Faculty of Medicine, Fukuoka University, Fukuoka, Japan
Background/Purpose: Only a few reports have focused on the rate of renal function deterioration in IgG4-related tubulointerstitial nephritis (IgG4-TIN). Some cases show acute or progressive…
Abstract Number: 1346 • 2017 ACR/ARHP Annual Meeting
Selective Effect of Rituximab on IgG4 Anti-CCP Autoantibodies in Rheumatoid Arthritis Patients
Mercedes Rincon^1,2, Sarah Kelso³, Janice Bunn⁴ and Sheldon Cooper⁵, ¹Department of Medicine/Rheumatology, University of Vermont, Burlingont VT, VT, ²Department of Medicine/Immunobiology, University of Vermont, Burlington, VT, ³Department of Medicine/Rheumatology, Univeristy of Vermont, Burlington, VT, ⁴Mathematics and Statistics, University of Vermont, Burlington, VT, ⁵Department of Medicine/Rheumatology, University of Vermont, Burlington, VT
Background/Purpose: Autoantibodies have long been recognized to be present in patients with RA, but it is more recent that autoantibodies against citrullinated proteins (anti-CCP) are…
Abstract Number: 1847 • 2017 ACR/ARHP Annual Meeting
The IgG4:IgG RNA Ratio in Peripheral Blood Perfectly Differentiates Active Disease from Remission in Granulomatosis with Polyangiitis. a New Disease Activity Marker?
Aram Al-Soudi^1,2,3, Marieke E. Doorenspleet⁴, Rebecca Esveldt⁵, Lot Burgemeister⁵, Liesbeth Hak⁵, Sander W. Tas⁶, Ronald F van Vollenhoven⁷, Paul L. Klarenbeek^5,8 and Niek de Vries^5,9, ¹Dept of Experimental Immunology, Academic Medical Center, Amsterdam, Netherlands, ²Dept of Clinical Immunology & Rheumatology, ARC | Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ³Dept of Genome Analysis, Academic Medical Center, Amsterdam, Netherlands, ⁴ARC | Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ⁵Clinical Immunology & Rheumatology, ARC | Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ⁶Department of Clinical Immunology & Rheumatology and Laboratory for Experimental Immunology, ARC | Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ⁷Amsterdam Rheumatology and Immunology Center, Amsterdam, Netherlands, ⁸Dept. of Experimental Immunology, Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ⁹Laboratory of Experimental Immunology, Academic Medical Center / University of Amsterdam, Amsterdam, Netherlands
Background/Purpose: Granulomatosis with Polyangiitis (GPA) is characterized by vasculitis in lungs, kidneys and the ear, nose and throat region. Regular monitoring and treatment adjustments are…
Abstract Number: 1882 • 2017 ACR/ARHP Annual Meeting
Thymus and Activation-Regulated Chemokine (TARC) As Biomarker for IgG4-Related Disease
Masataka Umeda^1,2, Tomoki Origuchi³, Shinya Kawashiri^1,4, Tomohiro Koga^1,5, Kunihiro Ichinose¹, Yushiro Endo¹, Sousuke Tsuji¹, Ayuko Takatani¹, Takashi Igawa¹, Toshimasa Shimizu¹, Shoichi Fukui^1,4, Remi Sumiyoshi¹, Ayako Nishino^1,6, Naoki Iwamoto¹, Mami Tamai¹, Hideki Nakamura¹ and Atsushi Kawakami¹, ¹Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, ²Medical Education Development Center, Nagasaki University Hospital, Nagasaki, Japan, ³Department of Rehabilitation Sciences, Nagasaki University, Nagasaki, Japan, ⁴Departments of Community Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, ⁵Center for Bioinformatics and Molecular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki City, Japan, ⁶Center for Comprehensive Community Care Education, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
Background/Purpose: TARC, also known as chemokine ligand 17 (CCR17), is expressed in the thymus and is produced by dendritic cells, endothelial cells, keratinocytes and fibroblasts.…
Abstract Number: 2093 • 2017 ACR/ARHP Annual Meeting
Comparison of Clinical and Laboratory Features of Patients with and without Allergic Conditions in IgG4-Related Disease: A Single-Center Experience in Japan
Takako Saeki, Tomoyuki Ito, Maasa Tamura, Seiichi Yoshikawa and Hajime Yamazaki, Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan
Background/Purpose: Although patients with IgG4-related disease (IgG4-RD) sometimes have accompanying allergic conditions, few data on the relationship between allergic conditions and IgG4-RD have been available.…