Abstracts tagged "IgG4 Related Disease"

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: 1385 • 2015 ACR/ARHP Annual Meeting
Efficacy and Tolerance of Rituximab in IgG4-Related Disease: A Retrospective Multicentric Study in 24 Patients
Mikael Ebbo¹, Aurélie Grados¹, Maxime Samson², Clarisse Carra-Dallière³, Agnieszka Pozdzik⁴, Pierre Labauge³, Sylvain Palat⁵, Jean-Marie Berthelot⁶, Jean-Loup Pennaforte⁷, Alain Wynckel⁸, Celine Lebas⁹, Thomas Quémeneur¹⁰, Karine Dahan¹¹, Jean-Jacques Boffa¹¹, Bertrand Godeau¹², Nicolas Limal¹², Franck Carbonnel¹³, Anne Herber¹⁴, Gaëlle Leroux¹⁵, Patrice Cacoub¹⁶, Alexis Mathian¹⁷, Eric Hachulla¹⁸, Nathalie Costedoat-Chalumeau¹⁹, Jean-Robert Harle²⁰ and Nicolas Schleinitz¹, ¹Internal Medicine, Aix-Marseille Université, AP-HM, Marseille, France, ²Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, ³Neurology, CHRU de Montpellier, Montpellier, France, ⁴Nephrology, Erasme Hospital, Cliniques Universitaires de Bruxelles, Bruxelles, Belgium, ⁵Service de Medecine Interne, CHU limoges, Limoges, France, ⁶Rhumatologie, CHU Nantes, Nantes, France, ⁷Internal Medicine, CHU de Reims, Reins, France, ⁸Nephrolohy, CHU de Reims, Reims, France, ⁹Nephrology, CHRU de Lille, Lille, France, ¹⁰CH Valenciennes, Valenciennes, France, ¹¹Nephrology, Tenon Hospital, Paris, France, ¹²Internal Medicine, Mondor Hospital, Creteil, France, ¹³Gastro-enterology, CHU Bicêtre, Le Kremlin Bicêtre, France, ¹⁴Gastro-enterology, CH de Chartres, Chartres, France, ¹⁵Department of Internal Medicine 2. Referal center for SLE/APS, CHU Pitié-Salpêtrière, Paris, France, ¹⁶Internal Medicine Department, University Hospital “Pitié-Salpêtrière”, “Pierre et Marie Curie Paris VI” University, Paris, France, ¹⁷Internal Medecine Department, Pitie-Salpetriere Hospital, Paris, France, ¹⁸FAI²R, Hôpital Claude Huriez, CHRU Lille, LILLE, France, ¹⁹Internal Medicine Department, Cochin Hospital, “René-Descartes Paris V” University, Paris, France, ²⁰Internal Medicine, Aix-Marseille Université, APHM, Marseille, France
Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition with characteristic histopathological lesions. Nearly all anatomic sites can be involved with a risk of organ…
Abstract Number: 1386 • 2015 ACR/ARHP Annual Meeting
Malignancy Prevalence Is Increased Among Patients before the Onset of IgG4-Related Disease
Zachary Wallace¹, John H. Stone², Hyon K. Choi³, Na Lu³ and Carly Wallace⁴, ¹Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, ²Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, ³Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ⁴Rheumatology Unit, Massachusetts General Hospital, Boston, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease of unclear etiology. Studies have suggested that patients with IgG4-RD may be at increased risk of…
Abstract Number: 1387 • 2015 ACR/ARHP Annual Meeting
Thoracic Paravertebral Masses and IgG4-Related Disease: Report of 8 Cases and Review of the Literature
Zachary Wallace¹, Sian Lim¹, John H. Stone², Micheal McInnis³ and Amita Sharma⁴, ¹Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, ²Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, ³Radiology Department, Thoracic Imaging, Massachusetts General Hospital, Boston, MA, ⁴Department of Radiology, Thoracic Imaging, Massachusetts General Hospital, Boston, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory condition that often leads to tumefactive lesions. We describe a common but under-recognized radiologic finding in this…
Abstract Number: 1991 • 2015 ACR/ARHP Annual Meeting
Large Vessel Involvement By IgG4-Related Disease
Cory Perugino¹, Zachary Wallace¹ and John H. Stone², ¹Rheumatology, Massachusetts General Hospital, Boston, MA, ²Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition of uncertain etiology. Reports have described inflammatory aortitis and peri-aortitis in the setting of retroperitoneal fibrosis…
Abstract Number: 2040 • 2015 ACR/ARHP Annual Meeting
T Follicular Helper like CD4+CXCR5+pd-1+ Cells and T Follicular Helper like Type 2 Cells Subsets Are Increased in 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 Bonotte¹⁵, Catherine Farnarier¹⁶, Jean-Robert Harle¹⁷, Nathalie Costedoat-Chalumeau⁷, Frederic Vely¹⁸ 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, ⁸Medecine Interne, 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, ¹⁵Department of Internal medicine and Clinicla Immunology Dijon University Hospital, Dijon, France, ¹⁶Laboratoire d'immunologie, Hopital de la Conception, Marseille, France, ¹⁷Internal Medicine, Aix-Marseille Université, APHM, Marseille, France, ¹⁸CIML, 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.…
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: 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: 2649 • 2013 ACR/ARHP Annual Meeting
Rituximab For The Treatment Of IgG4-Related Disease: A Prospective Clinical Trial
Mollie Carruthers¹, Mark Topazian², Arezou Khosroshahi³, Thomas Witzig⁴, Judith Oakley⁵, Philip Hart⁶, Lauren Kelly⁵, Lori Bergstrom⁶, Suresh Chari⁶ and John Stone⁷, ¹Rheumatology, Massachusetts General Hospital, Boston, MA, ²Gastroenterology, Mayo Clinic, Rochester, MA, ³Rheumatology, Emory University School of Medicine, Atlanta, GA, ⁴Hematology & Oncology, Mayo Clinic, Rochester, MN, ⁵Clinical Research Program, Massachusetts General Hospital, Boston, MA, ⁶Gastroenterology, Mayo Clinic, Rochester, MN, ⁷Rheumatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA
Background/Purpose: IgG4-related disease (IgG4-RD) is a multi-organ, fibro-inflammatory disorder. An open label pilot trial of rituximab (RTX) in IgG4-RD conducted at twocentershas enrolled 29 of…

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