Abstracts tagged "IgG4 Related Disease"

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: 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: 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: 2873 • 2017 ACR/ARHP Annual Meeting
DNA Microarray Analysis Identifies Nuclear Receptor Subfamily 4 Group a Member 2 (NR4A2) As a Novel Molecule Involved in the Pathogenesis of Sjogren’s Syndrome
Hiroyuki Takahashi, Hiroto Tsuboi, Hiromitsu Asashima, Hanae Kudo, Yuko Ono, Saori Abe, Yuya Kondo, Isao Matsumoto and Takayuki Sumida, Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan
Background/Purpose:Some reports on DNA microarray analysis in labial salivary glands (LSGs) of Sjögren’s syndrome (SS) and healthy controls (HCs) showed that the genes associated with…
Abstract Number: 1066 • 2017 ACR/ARHP Annual Meeting
Invention and Phenotypic Evaluation of Human IgG4-Knock-in Mice
Yoshie Gon, Hajime Yoshifuji, Koji Kitagori, Toshiki Nakajima, Kosaku Murakami, Ran Nakashima, Koichiro Ohmura and Tsuneyo Mimori, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
Background/Purpose: IgG4-related disease (IgG4-RD) is a disorder characterized by elevated serum IgG4 concentration and infiltration of IgG4-positive plasma cells into affected organs, however, the role…
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…

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