Abstracts tagged "IgG4 Related Disease"

Abstract Number: 892 • 2015 ACR/ARHP Annual Meeting
Autoimmune Associated Orbital Inflammatory Masses and Response to Immunosuppressive Therapy
Alina Casian¹, Shirish Sangle (joint first author)², Ritu Malaiya³, Pamela Lutalo⁴, Louise Nel⁵, Bina Menon⁶, Hema Varma⁷, Miles Stanford⁸ and David P. D'Cruz⁵, ¹Louise Coote Lupus Unit, Guy's and St Thomas' Hospital, London, UK, London, United Kingdom, ²Louise Coote Lupus Unit, Guy's and St. Thomas' Hospital, London, United Kingdom, ³Louise Coote Lupus Unit, Louise Coote Lupus Unit, St Thomas' Hospital, London, United Kingdom, ⁴Rheumatology, St. George's Hospital, London, United Kingdom, ⁵Louise Coote Lupus Unit, Guy's and St Thomas' Hospital, London, United Kingdom, ⁶Rheumatology, Guy's Hospital, London, United Kingdom, ⁷Radiology, Guy's and St. Thomas' Hospital, London, United Kingdom, ⁸Ophthalmology, Guy's and St. Thomas' Hospital, London, United Kingdom
Background/Purpose: To characterize a single centre retrospective case series of patients with orbital inflammatory masses associated with autoimmune diseases including granulomatosis with polyangiitis(GPA)(formerly Wegener’s granulomatosis),…
Abstract Number: 1105 • 2015 ACR/ARHP Annual Meeting
The IgG/IgG4 mRNA Ratio By Quantitative PCR Accurately Diagnoses IgG4-Related Disease and Predicts Treatment Response
Lowiek Hubers¹, Marieke Doorenspleet², Paulus Klarenbeek³, Emma Culver⁴, Lucas Maillette de Buy Wenniger⁵, Roger Chapman⁴, Stan Van de Graaf⁶, Joanne Verheij⁷, Thomas Van Gulik⁸, Frank Baas⁹, Ellie Barnes⁴, Ulrich Beuers¹ and Niek De Vries³, ¹Dept. of Gastroenterology & Hepatology and Tytgat Institute of Liver and Intestinal Research, Dept. of Gastroenterology & Hepatology and Tytgat Institute of Liver and Intestinal Research, Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ²Dept. of Clinical Immunology & Rheumatology, Amsterdam Rheumatology and immunology Center | Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ³Dept. of Clinical Immunology & Rheumatology, Amsterdam Rheumatology and immunology Center, Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ⁴Translational Gastroenterology Unit and NDM Oxford University, Translational Gastroenterology Unit and NDM Oxford University, John Radcliffe Hospital/Oxford University, Oxford, United Kingdom, ⁵1Dept. of Gastroenterology & Hepatology and Tytgat Institute of Liver and Intestinal Research, Dept. of Gastroenterology & Hepatology and Tytgat Institute of Liver and Intestinal Research, Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ⁶Dept. of Gastroenterology & Hepatology and Tytgat Institute of Liver and Intestinal Research,, Dept. of Gastroenterology & Hepatology and Tytgat Institute of Liver and Intestinal Research, Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ⁷Dept. of Pathology, Dept. of Pathology, Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ⁸Dept. of Surgery, Dept. of Surgery, Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands, ⁹Dept. of Genome Analysis, Academic Medical Center/University of Amsterdam, Amsterdam, Netherlands
Background/Purpose : IgG4-associated cholangitis (IAC) and autoimmune pancreatitis (AIP) are major manifestations of IgG4-related disease (IRD). Misdiagnosis and inadequate treatment are common since IAC and…
Abstract Number: 1379 • 2015 ACR/ARHP Annual Meeting
High Serum IgG4 Concentration Is a Risk Factor for Relapse in IgG4-Related Disease
Sho Sendo¹, Jun Saegusa¹, Akira Onishi², Ikuko Naka¹, Kosaku Tsuda¹, Takaichi Okano¹, Kengo Akashi¹, Soshi Takahashi¹, Miwa Nishida¹, Keisuke Nishimura¹, Yo Ueda¹, Yoshinori Kogata³, Goichi Kageyama¹, Goh Tsuji⁴, Shunichi Kumagai⁵ and Akio Morinobu¹, ¹Department of Rheumatology, Kobe University Hospital, Kobe, Japan, ²Department for Rheumatology, Kobe University Hospital, Kobe, Japan, ³Department of Clinical Pathology and Immunology, Kobe University Hospital, Kobe, Japan, ⁴Center for Rheumatic disease, Shinko Hospital, Kobe, Japan, ⁵Center for Rheumatic Disease, Shinko Hospital, Kobe, Japan
Background/Purpose: IgG4-related disease (IgG4-RD) is a recently recognized systemic fibro-inflammatory disease of unknown etiology with multi-organ involvement. Although the organ involvement in IgG4-RD usually improve…
Abstract Number: 1380 • 2015 ACR/ARHP Annual Meeting
Characterization of Lymphocytes Subsets in Peripheral Blood of Untreated IgG4-Related Disease Patients
Aurélie Grados¹, Mikael Ebbo¹, Christelle Piperoglou², Matthieu Groh³, Alexis Regent⁴, Maxime Samson⁵, Benjamin Terrier⁶, Nathalie Morel⁷, Sylvain Audia⁵, Francois Maurier⁸, Julie Graveleau⁹, Mohamed Hamidou¹⁰, Amandine Forestier¹¹, Sylvain Palat¹², Emanuelle Bernit¹, Gilles Kaplanski¹³, Frederique Retornaz¹⁴, Bernard Bonotte⁵, Catherine Farnarier¹⁵, Jean-Robert Harle¹⁶, Nathalie Costedoat-Chalumeau⁷, Frederic Vely¹⁷ and Nicolas Schleinitz¹, ¹Internal Medicine, Aix-Marseille Université, AP-HM, Marseille, France, ²Immunology, CIML, AP-HM, Marseille, France, ³National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, ⁴Service de médecine interne, Hôpital Cochin, Paris, France, ⁵Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, ⁶Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Paris, France, ⁷Internal Medicine Department, Cochin Hospital, “René-Descartes Paris V” University, Paris, France, ⁸HP Metz Belle Isle Hospital, Department of Internal Medicine, Metz, France, ⁹Medecine Interne Hotel Dieu Nantes, Nantes, France, ¹⁰Internal Medicine Department, Nantes University Hospital, Nantes, France, ¹¹Internal Medicine, Groupe Hospitalier Mutualiste de Grenoble, Grenoble, France, ¹²Service de Medecine Interne, CHU limoges, Limoges, France, ¹³Internal Medicine hopital conception, Aix-Marseille Université, Marseille, France, ¹⁴Conseil General 13 cellule recherche, Marseille, France, ¹⁵Laboratoire d'immunologie, Hopital de la Conception, Marseille, France, ¹⁶Internal Medicine, Aix-Marseille Université, APHM, Marseille, France, ¹⁷CIML, Laboratoire d'Immunologie Conception AP-HM, Aix-Marseille université, Marseille, France
Background/Purpose: IgG4-related disease (IgG4-RD) is associated with characteristic pathological changes including lymphoplasmocytic infiltration with abundant IgG4 positive plasma cells, storiform fibrosis and obliterative phlebitis. The…
Abstract Number: 1381 • 2015 ACR/ARHP Annual Meeting
The Relationship Between Serum Cholinesterase, Number of Organ Involvement and Fibrotic Markers in Japanese Patients with IgG4-Related Disease
Hirofumi Nishikawa¹, Yoshinori Taniguchi¹, Yoshiko Shimamura¹, Kosuke Inoue¹, Taro Horino¹, Susumu Nishiyama², Yasuhiko Yoshinaga² and Yoshio Terada¹, ¹Kochi University, Nankoku, Japan, ²Kurashiki Medical Center, Kurashiki, Japan
Background/Purpose: To evaluate the relationship between cholinesterase, number of organ involvement and serum fibrotic markers in Japanese patients with IgG4-related disease (IgG4-RD). Methods: The clinical…
Abstract Number: 1382 • 2015 ACR/ARHP Annual Meeting
Clinical and Serum IgG4 Characteristics of a Unique British Columbian IgG4-Related Disease Cohort
Luke Chen¹, Andre Mattman², Sujin Park³, Brian Skinnider⁴, Graham Slack⁴ and Mollie Carruthers⁵, ¹Hematology, University of British Columbia, Vancouver, BC, Canada, ²Medical Biochemistry, University of British Columbia, Vancouver, BC, Canada, ³University of British Columbia, Vancouver, BC, Canada, ⁴Pathology, University of British Columbia, Vancouver, BC, Canada, ⁵Rheumatology, University of British Columbia, Vancouver, BC, Canada
Background/Purpose: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder that is known to have protean manifestations and variably elevated serum IgG4 concentrations. We present the serum…
Abstract Number: 1384 • 2015 ACR/ARHP Annual Meeting
Comparison of IgG4-Related and Non-IgG4-Related Retroperitoneal Fibrosis; A 12 Year Retrospective Study
Piyush Poddar^1,2, Steven Billings³, Leonard H. Calabrese⁴ and Carmen E. Gota⁵, ¹Rheumatology, Sanford Medical center, Bismarck, ND, ²Rheumatology, Cleveland Clinic, Cleveland, OH, ³Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, ⁴Rheumatic & Immunologic Dis, Cleveland Clinic Foundation, Cleveland, OH, ⁵Orthopedic and Rheumatologic Institute, Cleveland Clinic, Cleveland, OH
Comparison of IgG4-related and Non-IgG4-related Retroperitoneal fibrosis; a 12 year Retrospective Study Background/Purpose: Retroperitoneal fibrosis (RPF) is a rare disease characterized by a progressive proliferation…
Abstract Number: 2805 • 2014 ACR/ARHP Annual Meeting
IgG4-Related Disease: Baseline Features in 100 Patients with Biopsy-Proven Disease
Zachary Wallace¹, Vikram Deshpande², Hamid Mattoo³, Vinay Mahajan⁴, Mollie Carruthers¹, Maria Kulikova⁵, Shiv Pillai⁶ and John H. Stone⁷, ¹Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, ²Pathology, Massachusetts General Hospital, Boston, MA, ³Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ⁴Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ⁵Massachusetts General Hospital, Boston, MA, ⁶Oncology, Massachusetts General Hospital, Boston, MA, ⁷Rheumatology, Massachusetts General Hospital, Boston, MA
Background/Purpose IgG4-related disease (IgG4-RD) has emerged as an immune-mediated disease that links multiple fibro-inflammatory conditions through common pathology and pathophysiologic mechanisms. Most of the literature…
Abstract Number: 2806 • 2014 ACR/ARHP Annual Meeting
Characteristic Phenotype of Peripheral Blood Lymphocytes in Patients with IgG4-Related Disease, Comparing to Primary Sjögren Syndrome and Healthy Controls
Shintaro Hirata, Shingo Nakayamada, Satoshi Kubo, Maiko Yoshikawa, Naoki Yunoue, Kazuhisa Nakano, Kunihiro Yamaoka, Kazuyoshi Saito and Yoshiya Tanaka, The First Department of Internal Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan
Background/Purpose : IgG4-related disease (IgG4-RD) is a systemic disease that is characterized by the infiltration of IgG4-positive plasma cells and T cells into various organs.…
Abstract Number: 1650 • 2014 ACR/ARHP Annual Meeting
Prevalence and Prognostic Implications of IgG4 in Membranous Lupus Nephritis
David Herrera Van Oostdam¹, Marco Ulises Martinez-Martinez¹, Cuauhtémoc Oros-Ovalle², David Martínez-Galla², Gerardo Tonatiu Jaimes-Piñón¹, Natalia Alemán-Sánchez² and Carlos Abud-Mendoza¹, ¹Unidad de Investigaciones Reumatológicas, Hospital Central & Facultad de Medicina, Universidad Autónoma de San Luis Potosí, San Luis Potosí, Mexico, ²Patología, Hospital Central & Facultad de Medicina, Universidad Autónoma de San Luis Potosí, San Luis Potosí, Mexico
Background/Purpose: Patients with membranous lupus nephritis (MLN) have increased risk of thrombosis and different prognosis than other classes of lupus nephritis (LN). Previous studies demonstrated…
Abstract Number: 1239 • 2014 ACR/ARHP Annual Meeting
Is Lymphocytic Sialadenitis IgG4-Related?
Nathalie Shehwaro¹, Murielle Hourseau², Thomas Papo³ and Karim Sacre⁴, ¹Internal Medicine, University Paris-7, APHP, Bichat Hospital, Paris, France, ²Pathology, University Paris-7, APHP, Bichat Hospital, Paris, France, ³Internal Medicine, Bichat Hospital, Paris, Paris, France, ⁴Internal Medicine, University Paris-7, INSERM U699, APHP, Bichat Hospital, Paris, France
Background/Purpose: To assess the prevalence of IgG4-related disease among patients with lymphocytic sialadenitis on labial salivary gland biopsy. Methods: All labial salivary gland biopsies (LSGB)…
Abstract Number: 1238 • 2014 ACR/ARHP Annual Meeting
Proportion of Peripheral Plasmacytoid Dendritic Cells and Plasmablasts Reflects Disease Activity in IgG4-related Disease
Mitsuhiro Akiyama¹, Katsuya Suzuki¹, Yoshiaki Kassai², Takahiro Miyazaki², Rimpei Morita³, Akihiko Yoshimura³ and Tsutomu Takeuchi¹, ¹Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan, ²Inflammation Drug Discovery Unit, Takeda Pharmaceutical Company Limited, Kanagawa, Japan, ³Department of Microbiology and Immunology, Keio University School of Medicine, Tokyo, Japan
Background/Purpose Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disease with multi-organ system involvement. Affected patients frequently have a history of bronchial asthma and allergic rhinitis. The reported pathogenesis of IgG4-RD describes the clear involvement of excessive Th2 cells and regulatory immune reaction in addition to plasma cells 1). However, peripheral immune cell phenotype, which reflects disease status, has not been comprehensively evaluated. Our aim was to definitively determine peripheral blood cell abnormalities and their correlation with disease activity in patients with IgG4-RD.Methods Peripheral blood samples were obtained from active untreated IgG4-RD patients (n=11) and healthy controls (n=16). Comprehensive immunophenotyping assay with information on activation status was done by multi-color flow cytometry, and the proportion of peripheral blood mononuclear cells (PBMCs), including T cells (naïve/memory, Th1/2/17, Treg, and Tfh), B cells (naïve/memory, plasmablast, Breg), monocytes (classical, intermediate, non-classical) and dendritic cells (myeloid, plasmacytoid), and their activity status were precisely defined. Disease activity was measured using the IgG4-RD responder index (RI). Statistical analysis was done using the Mann-Whitney U test and Spearman rank correlation coefficient test.Results The proportion of plasmablasts (CD19+CD20-CD27+CD38+), memory Th2 cells (CD3+CD4+CXCR3-CCR6-CD45RA-), Tregs (CD3+CD4+CD25+CD127low), Tfh (CD3+CD4+CXCR5+), and mDCs (CD3-CD19-CD14-HLA-DR+CD1c+CD303-) in peripheral blood was significantly increased in IgG4-RD patients compared with HC, whereas the proportion of pDCs (CD3-CD19-CD14-HLA-DR+CD1c-CD303+）was significantly decreased. Interestingly, the proportion of pDCs in total DCs was negatively correlated with IgG4-RD RI (r=-0.778, p=0.005) while the proportion of plasmablasts in CD19+cells was positively correlated with RI (r=0.701, p=0.016). Further, the increased proportion of plasmablasts was positively correlated with serum IgG4 level (r=0.718, p=0.013) while the decreased proportion of pDCs tended to be negatively correlated with the number of affected organs (r=-0.518, p=0.061). Conclusion Our comprehensive analysis identified distinct proportional changes in PBMCs in IgG4-RD. In particular, the decrease in pDCs and increase in plasmablasts were strongly linked with disease activity. These combined measurements are expected to be clinically useful surrogate cell markers. This newly identified decrease in circulating pDCs may be involved in the pathogenesis in IgG4-RD via the recently described role in the enhancement of Th2 response 2).References: 1) Stone JH et.al. N Engl J Med 2012;366:539-51 2) Maazi H et.al. Allergy 2013;68:695-701
Abstract Number: 1236 • 2014 ACR/ARHP Annual Meeting
IgG4 Immunostaining Is Common but Not Specific in Orbitbal Inflammatory Diseases
James T. Rosenbaum^1,2, Amanda Wong³, Patrick Stauffer³, Megan Troxell⁴, Donald Houghton⁵, Dongseok Choi⁶, Christine Harrington⁷, David Wilson⁸, Hans Grossniklaus⁹, Roger Dailey³, John Ng³, Eric Steele³, Patrick Yeatts¹⁰, Peter Dolman¹¹, Valerie White¹¹, Craig Czyz¹², Jill Foster¹², Deepak Edward¹³, Hind Alkatan¹³, Don Kikkawa¹⁴, Bobby Korn¹⁵, Dinesh Selva¹⁶, Gerald Harris¹⁷, Michael Kazim¹⁸, Payal Patel¹⁸ and Stephen R. Planck¹⁹, ¹OHSU, Portland, OR, ²Arthritis and Rheumatic diseases, Oregon Health and Science University, Portland, OR, ³Oregon Health & Science University, Portland, OR, ⁴Pathology, Oregon Health and Science University, Portland, OR, ⁵Pathology, Oregon Health & Science University, Portland, OR, ⁶Casey Eye Institute, Oregon Health and Science University, Portland, OR, ⁷Integrated Genomics, Oregon Health & Science University, Portland, OR, ⁸Casey Eye Institute/Ophthalmology, Oregon Health & Science University, Portland, OR, ⁹Ophthalmology, Emory University, Atlanta, GA, ¹⁰Ophthalmology, Wake Forest University, Winston-Salem, NC, ¹¹University of British Columbia, Vancouver, BC, Canada, ¹²Ohio State Univeristy, Columbus, OH, ¹³King Khaled Eye Hospital, Riyadh, Saudi Arabia, ¹⁴Ophthalmology, University of California, San Diego, San Diego, CA, ¹⁵University of California, San Diego, San Diego, CA, ¹⁶Royal Adelaide Hospital, Adelaide, Australia, ¹⁷Medical College of Wisconsin, Milwaukee, WI, ¹⁸Columbia University, New York City, NY, ¹⁹Casey Eye Inst/Ophthalmology, Oregon Health & Science University, Portland, OR
Background/Purpose: IgG4-related disease is an emerging clinical entity which frequently involves tissue within the orbit. In order to appreciate the implications of IgG4 immunostaining, we…
Abstract Number: 1235 • 2014 ACR/ARHP Annual Meeting
Recovery of Renal Function after Corticosteroid Therapy for IgG4-Related Kidney Disease
Takako Saeki¹, Mitsuhiro Kawano², Ichiro Mizushima², Motohisa Yamamoto³, Yoshifumi Ubara⁴, Hitoshi Nakashima⁵, Yoko Wada⁶, Tomoyuki Ito⁷, Hajime Yamazaki⁷, Ichiei Narita⁶ and Takao Saito^5,8, ¹Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan, ²Kanazawa University Hospital, Kanazawa, Japan, ³Sapporo Medical University School of Medicine, Sapporo, Japan, ⁴Toranomon Hospital, Tokyo, Japan, ⁵Div of Nephrol & Rheumatol, Dept of Int Med, Faculty of Medicine, Fukuoka University, Fukuoka, Japan, ⁶Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan, ⁷Nagaoka Red Cross Hospital, Nagaoka, Japan, ⁸General Medical Research Center, Faculty of Medicine, Fukuoka University, Fukuoka, Japan
Background/Purpose: In our earlier study of IgG4-related kidney disease (IgG4-RKD), we found that renal dysfunction, which was mostly attributable to IgG4-related tubulointerstitial nephritis, was significantly…
Abstract Number: 784 • 2013 ACR/ARHP Annual Meeting
IgG4+ Plasmablasts Are A Novel Biomarker In IgG4-Related Disease
Mollie Carruthers¹, Hamid Mattoo², Zachary S. Wallace³, Vinay Mahajan⁴, Shiv Pillai⁵ and John H. Stone¹, ¹Rheumatology, Massachusetts General Hospital, Boston, MA, ²Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ³Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, ⁴Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ⁵Oncology, Massachusetts General Hospital, Boston, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is an immune-mediated disorder that responds to B cell depletion with rituximab (RTX). We detected IgG4+ plasmablasts in the sera of…

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