Abstracts Archive - Page 1338 of 2607 - ACR Meeting Abstracts

Abstract Number: 2276 • 2018 ACR/ARHP Annual Meeting
Risk of Hemorrhagic Strokes in Patients with Adenosine Deaminase 2 Deficiency
Patrycja M. Hoffmann¹, Amanda Ombrello¹, Deborah L. Stone², Dean Follmann³, Karyl Barron⁴, Anne Jones⁵, Tina Romeo¹, Camilo Toro⁶, Ariane Soldatos⁷, Arielle Hay⁸, Qing Zhou⁹, Ivona Aksentijevich¹⁰ and Daniel L. Kastner⁵, ¹NHGRI, National Institutes of Health, Bethesda, MD, ²Inflammatory Disease Section, NHGRI/NIH, Bethesda, MD, ³NIAID, Bethesda, MD, ⁴National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁵Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ⁶NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ⁷NINDS, National Institutes of Health, Bethesda, MD, ⁸Nicklaus Children’s Hospital, Miami, FL, ⁹China, ¹⁰Metabolic, Cardiovascular, and Inflammatory Disease Genomics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD
Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive condition characterized by recurrent fevers, early-onset ischemic strokes, livedo racemosa, polyarteritis nodosa, portal hypertension,…
Abstract Number: 2277 • 2018 ACR/ARHP Annual Meeting
Colchicine: An Effective Treatment Option for Unclassified Autoinflammatory Diseases in Children
Jasmin B. Kuemmerle-Deschner¹, Anna Lena Schock², Sandra Hansmann³ and Susanne Benseler⁴, ¹Department of Pediatrics, Division of Rheumatology, University Hospital Tuebingen, Germany, Tuebingen, Germany, ²Department of Pediatrics, Division of Rheumatology, University Hospital Tuebingen, Tuebingen, Germany, Tuebingen, Germany, ³Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tuebingen, Tuebingen, Germany, Tuebingen, Germany, ⁴Rheumatology, Department of Paediatrics, Alberta Children's Hospital, University of Calgary, Alberta, Canada, Calgary, AB, Canada
Background/Purpose: Interleukin-1 (IL-1) inhibition was found to be an effective, yet very expensive treatment option for children with clinically and genetically defined autoinflammatory diseases (AID).…
Abstract Number: 2278 • 2018 ACR/ARHP Annual Meeting
Cryopyrinopathy across Generations: Longterm Disease Outcome
Sarka Fingerhutova¹, Jana Franova², Eva Hlavackova³, Eva Jancova⁴, Leona Prochazkova⁵, Marketa Tesarova⁶ and Pavla Dolezalová⁷, ¹Paediatric Rheumatology Unit, General University Hospital in Prague and 1st Faculty of Medicine, Charles University, General University Hospital in Prague and 1st Faculty of Medicine, Prague, Czech Republic, ²Department of Paediatric Rheumatology, University Hospital Brno, Brno, Czech Republic, Brno, Czech Republic, ³Department of Allergology and Immunology, St Ann´s Hospital Brno, University Hospital, Brno, Czech Republic, Brno, Czech Republic, ⁴Department of Nephrology, General University Hospital in Prague and 1st Faculty of Medicine, Prague, Czech Republic, ⁵Department of Rheumatology, St Ann´s Hospital Brno, University Hospital, Brno, Czech Republic, Brno, Czech Republic, ⁶Laboratory for study of Mitochondrial Disorders, Department of Paediatrics and Adolescent Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ⁷Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic
Background/Purpose: Cryopyrinopathies are autoinflammatory disorders (AID) caused by mutations of NLRP3 gene that lead to interleukin-1 (IL-1) overproduction with the clinical picture of periodic fever.…
Abstract Number: 2279 • 2018 ACR/ARHP Annual Meeting
Therapeutic Value of Canakinumab in Patients with Yao Syndrome
Qingping Yao, Rheumatology, Allergy and Immunology, Stony Brook University Hospital, Stony Brook, NY; Rheumatology, Allergy, and Immunology, Stony Brook University School of Medicine, Stony Brook, NY
Background/Purpose: Yao syndrome (YAOS, OMIM 617321), formerly termed nucleotide-binding, oligomerization domain 2(NOD2)-associated autoinflammatory disease, is characterized by periodic fever, dermatitis, arthritis, and swelling of the…
Abstract Number: 2280 • 2018 ACR/ARHP Annual Meeting
Transcriptomic Analysis of Hidradenitis Suppurativa Skin Demonstrates Dysregulation of Antimicrobial Proteins and Inflammatory Pathways
Victoria Shanmugam¹, Derek Jones², Matthew Bendall³ and Keith Crandall⁴, ¹Rheumatology, The George Washington University, Washington, DC, ²The George Washington University, Washington, DC, ³Department of Microbiology, Immunology and Tropical Medicine, The George Washington University, Washington, DC, ⁴Department of Biology, The George Washington University, Washington, DC
Background/Purpose: Hidradenitis suppurativa (HS) is a chronic, recurrent, inflammatory disease of the apocrine sweat glands. The purpose of the current study was to identify transcripts…
Abstract Number: 2281 • 2018 ACR/ARHP Annual Meeting
Treatment Choices and Response Rates of SAPHO Syndrome: Single Center Case Series
Abdulsamet Erden¹, Mustafa Ekici², Alper Sari³, Berkan Armagan³, Levent Kilic³, Sule Apras Bilgen¹, Ali Akdogan¹, Omer Karadag¹, Sedat Kiraz³ and Ihsan Ertenli¹, ¹Rheumatology, Hacettepe University, Faculty of Medicine, Ankara, Turkey, ²Hacettepe University Faculty of Medicine, Ankara, Turkey, ³Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey
Background/Purpose: SAPHO Syndrome is a chronic disease with bone, joint and skin involvement characterized by synovitis, acne, pustulosis, hyperostosis, osteitis. Treatment decisions of SAPHO syndrome…
Abstract Number: 2282 • 2018 ACR/ARHP Annual Meeting
Measurement of the Pro-Coagulant Activity of Microparticles in Patients with Inflammatory Rheumatic Diseases: Prospective Study
Christine Mekhail¹, Xavier Guillot², Clément Prati³, Philippe Saas⁴, Guillaume Mourey⁵ and Daniel Wendling⁶, ¹Rheumatology, CHRU, Besançon, France, ²PEPITE EA 4267, Bourgogne-Franche-Comté university, Besançon, France, ³Rheumatology, University Hospital - Bourgogne Franche Comté University, Besancon, France, ⁴INSERM UMR1098, Besançon university hospital, Besançon, France, ⁵EFS, Besançon, France, ⁶Rheumatology, University Hospital - Bourgogne Franche Comté University, Besançon, France
Background/Purpose: Microparticles (MPs) are small membrane-bound vesicles that arise from activated and dying cells. Although the majority of MPs in the blood originate from platelets,…
Abstract Number: 2283 • 2018 ACR/ARHP Annual Meeting
Incidental Steroid Use May Worsen Outcomes in Patients with Heparin Induced Thrombocytopenia in the ICU Setting
Roy Souaid¹ and Connie Lu², ¹Internal medicine, Brown University, Providence, RI, ²Medical school, Brown university, Providence, RI
Background/Purpose: Heparin induced thrombocytopenia (HIT) is the destruction of platelets in patients exposed to heparin products. While there are two types of HIT, the term…
Abstract Number: 2284 • 2018 ACR/ARHP Annual Meeting
Safety and Efficacy of Lenabasum in Refractory Skin-Predominant Dermatomyositis Subjects Treated on an Open-Label Extension of Trial JBT101-DM-001
Victoria P. Werth^1,2, David Pearson^1,2, Joyce Okawa^1,2, Rui Feng³, Josef Concha^1,2, Basil Patel^1,2, Emily Hejazi^1,2, Caitlin Cornwall⁴, Scott Constantine⁴ and Barbara White⁴, ¹University of Pennsylvania, Philadelphia, PA, ²Philadelphia Veterans Affairs Medical Center, Philadelphia, PA, ³University of Pennsylvania, Philadelphia', PA, ⁴Corbus Pharmaceuticals, Inc., Norwood, MA
Background/Purpose: Lenabasum is a synthetic, non-immunosuppressive, selective cannabinoid receptor type 2 agonist that activates resolution of innate immune responses. Lenabasum had acceptable safety and tolerability…
Abstract Number: 2285 • 2018 ACR/ARHP Annual Meeting
Factors Associated with Corticosteroid Discontinuation, Complete Clinical Response and Remission in Patients with Juvenile Dermatomyositis
Takayuki Kishi^1,2, William Warren-Hicks³, Nastaran Bayat¹, Ira Targoff⁴, Terri H Finkel⁵, Ellen Goldmuntz⁶, Michael Henrickson⁷, Bianca Lang⁸, Andrew Mammen⁹, Lauren M. Pachman¹⁰, Murray Passo¹¹, Terrance P. O'Hanlon¹, Frederick W. Miller¹, Michael Ward⁹ and Lisa G. Rider¹, ¹Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, ²Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan, ³EcoStat, Inc., Mebane, NC, ⁴VA Medical Center, University of Oklahoma Health Sciences Center and Oklahoma Medical Research Foundation, Oklahoma City, OK, ⁵Department of Pediatrics, Nemours Children's Health System/ Nemours Children's Hospital, Orlando, FL, ⁶NIAID, NIH, Bethesda, MD, ⁷Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁸Department of Pediatrics, IWK Health Centre and Dalhousie University, Halifax, NS, Canada, ⁹National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ¹⁰Cure JM Program of Excellence in Juvenile Myositis Research, Stanley Manne Children’s Research Institute, affiliated with Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, ¹¹Division of Rheumatology PTD, Medical University of South Carolina, Charleston, SC
Background/Purpose: We examined patients in a large juvenile dermatomyositis (JDM) registry for frequency of and factors associated with final corticosteroid discontinuation (Steroid DC), complete clinical…
Abstract Number: 2286 • 2018 ACR/ARHP Annual Meeting
Myositis and Fasciitis By Magnetic Resonance Imaging in Recent-Onset Polymyalgia Rheumatica and Effect of Tocilizumab Therapy
Jean Patrick Laporte¹, Florent Garrigues², Anais Huwart², Sandrine Jousse-Joulin³, Thierry Marhadour¹, Dewi Guellec³, Divi Cornec⁴, Valérie Devauchelle-Pensec³ and Alain Saraux³, ¹CHU Brest, Brest, France, ²Radiology, CHU Brest, Brest, France, ³Rheumatology, CHU Brest, Brest, France, ⁴Rheumatology and UMR1227, Lymphocytes B et Autoimmunité, CHU Brest, Brest, France
Background/Purpose: To assess the prevalence of myofascial inflammatory lesions visible by magnetic resonance imaging (MRI) and their changes after tocilizumab therapy in active polymyalgia rheumatica…
Abstract Number: 2287 • 2018 ACR/ARHP Annual Meeting
Interim Results of an Open-Label Study Assessing Efficacy and Safety of Adrenocorticotropic Hormone Gel for Treatment of Refractory Cutaneous Manifestations of Dermatomyositis
Anthony Fernandez, Dermatology and Pathology, Cleveland Clinic, Cleveland, OH
Background/Purpose: Cutaneous dermatomyositis (DM) is often refractory to multiple medications, suggesting better treatments are needed. Adrenocorticotropic hormone gel is a repository corticotropin injection that is…
Abstract Number: 2288 • 2018 ACR/ARHP Annual Meeting
Preexisting Anti-Acetylcholine Receptor Autoantibodies and B Cell Lymphopenia Are Associated with the Development of Myositis in Thymoma Patients Treated with Avelumab, an Immune Checkpoint Inhibitor Targeting Programmed Death-Ligand 1
Andrew Mammen¹, Arun Rajan², Katherine Pak¹, Tanya Lehky³, Livia Casciola-Rosen⁴, Renee Donahue², Lauren Lepone², Anastasia Zekeridou⁵, Sean Pittock⁵, Raffit Hassan², Jeffrey Schlom² and James Gulley², ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ²NCI, NIH, Bethesda, MD, ³NINDS, NIH, Bethesda, MD, ⁴Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ⁵Neurology, Mayo Clinic, Rochester, MN
Background/Purpose: Immune checkpoint inhibitors (ICIs) enhance the immune response against tumors but may also trigger immune-related adverse events (IRAEs). Myositis following receipt of ICIs is…
Abstract Number: 2289 • 2018 ACR/ARHP Annual Meeting
Granulomatous Myositis: Heterogeneity and Response to Treatment
Yannick Dieudonné¹, Yves Allenbach², Olivier Benveniste³, Sarah Leonard-louis⁴, Baptiste Hervier², Kuberaka Mariampillai³, Beatrice Lannes⁵, Daniel Wendling⁶, Christan Von Frenckell⁷, Nicolas Poursac⁸, Emmanuel Mortier⁹, Christian Lavigne¹⁰, Olivier Hinschberger¹¹, Julie Magnant¹², Bernard Geny¹³, Jean Sibilia¹⁴ and Alain Meyer¹⁵, ¹Département d’Immunologie Clinique et Médecine Interne, Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, STRASBOURG, France, ²Department of Internal Medicine and Clinical Immunology and Inflammation-Immunopathology-Biotherapy Department (I2B), Pitié-Salpêtrière University Hospital, Assistance Publique-Hôpitaux de Paris, East Paris Neuromuscular Diseases Reference Center, Paris, France, ³Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Neuro-Musculaires Paris Est, Assistance Publique – Hôpitaux de Paris (AP-HP), Hôpital Universitaire Pitié-Salpêtrière, Paris, France, ⁴Département de Neuropathologie, Centre de Référence des Maladies Neuro-Musculaires Paris Est, Assistance Publique – Hôpitaux de Paris (AP-HP), Hôpital Universitaire Pitié-Salpêtrière, Paris, France, ⁵Département de Pathologie, Hôpitaux Universitaires de Strasbourg, STRASBOURG, France, ⁶Rheumatology, University Hospital - Bourgogne Franche Comté University, Besançon, France, ⁷Département de Rhumatologie, Hôpital Universitaire Sart-Tilman, Liège, Belgium, ⁸9Departement de Rhumatologie, Hôpital Universitaire de Bordeaux, Bordeaux, France, ⁹Departement de Médicine Interne, Hôpital Universitaire Louis Mourier, Colombes, France, ¹⁰Departement de Médicine Interne et Maladies Vasculaires, Hopital Universitaire d'Angers, Angers, France, ¹¹Departement de Médicine Interne, Hôpital Emile Muller, Mulhouse, France, ¹²Departement de Médicine Interne, Hôpital Universitaire de Tours, Tours, France, ¹³Institut de Physiologie EA 3072, Service de Physiologie et d’Explorations Fonctionnelles, Hôpitaux Universitaires de Strasbourg, Strasbourg, France, ¹⁴Département de Rhumatologie, Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France, ¹⁵15Département de Rhumatologie, Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
Background/Purpose: Granuloma in the muscle can be found in patients with myopathy. This finding has alternatively been interpreted as either sarcoid myopathy or other myositis…
Abstract Number: 2290 • 2018 ACR/ARHP Annual Meeting
Long Term Follow-up Results of Myositis Patients Treated with H. P. Acthar Gel
Didem Saygin¹, Galina Marder², Chester V. Oddis³, Siamak Moghadam-Kia⁴, Preeya Nandkumar⁵, Zengbiao Qui⁶, Diane Koontz⁷ and Rohit Aggarwal⁸, ¹Internal Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, ²Rheumatology, Northwell Health, Manhasset, NY, ³Division of Rheumatology and Clinical Immunology, Department of Medicine, Unviersity of Pittsburgh/University of Pittsburgh Medical Center, Pittsburgh, PA, ⁴Rheumatology, University of Pittsburgh, Pittsburgh, PA, ⁵Department of Medicine-- Division of Rheumatology, Northwell Health, GREAT NECK, NY, ⁶Rheumatology, University of Pittsburgh Medical Center, Pittsburgh, PA, ⁷Internal Medicine Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, ⁸Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh/University of Pittsburgh Medical Center, Pittsburgh, PA
Background/Purpose: Although HP Acthar gel is a purified, injectable formulation of full-length adrenocorticotropic hormone approved by the FDA for use in myositis, peer-reviewed data is…

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