Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 0539 • ACR Convergence 2021
Vgll3-transgenic Autoimmune Mice Display Features of Cutaneous Fibrosis
Mehrnaz Gharaee-Kermani¹, Allison Billi², Marisa Hildebrandt², Jacob Martens³, Rachael Wasikowski², Joanne (Michelle) Kahlenberg¹ and Johann Gudjonsson², ¹Internal Medicine - Division of Rheumatology and Department of Dermatology, University of Michigan, Ann Arbor, MI, ²Department of Dermatology, University of Michigan, Ann Arbor, MI, ³Graduate Program in Immunology, University of Michigan, Ann Arbor, MI, USA, Ann Arbor, MI
Background/Purpose: Fibrosis is characterized by collagen deposition, fibro/myofibroblast accumulation, and extracellular matrix remodeling and can lead to disfiguring skin changes. In cutaneous lupus, scar formation…
Abstract Number: 1454 • ACR Convergence 2021
RHAPSODY: Rilonacept, an IL-1α and IL-1β Trap, Resolves Pericarditis Episodes and Reduces Risk of Recurrence in a Phase 3 Trial of Patients with Recurrent Pericarditis
Allan Klein¹, Massimo Imazio², Paul Cremer¹, Antonio Brucato³, Antonio Abbate⁴, Fang Fang⁵, Antonella Insalaco⁶, Martin LeWinter⁷, Basil S. Lewis⁸, David Lin⁹, Sushil A. Luis¹⁰, Stephen J. Nicholls¹¹, Arian Pano⁵, Alistair Wheeler¹², Liangxing Zou⁵ and John F Paolini⁵, ¹Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, ²University Cardiology, Cardiovascular and Thoracic Department, AOU Città della Salute e della Scienza di Torino, Torino, Italy, ³Department of Biomedical and Clinical Science, University of Milano, Fatebenefratelli Hospital, Milan, Italy, ⁴VCU Pauley Heart Center, Virginia Commonwealth University, Richmond, VA, ⁵Kiniksa Pharmaceuticals Corp., Lexington, MA, ⁶Department of Pediatric Medicine, Division of Rheumatology, Bambino Gesù Children's Hospital, Rome, Italy, ⁷Cardiology Unit, The University of Vermont Medical Center, The University of Vermont, Burlington, VT, ⁸Cardiovascular Clinical Research Institute, Lady Davis Carmel Medical Center, Haifa, Israel, ⁹Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, ¹⁰Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, NY, ¹¹MonashHeart, Department of Cardiology, Monash University, Clayton, Australia, ¹²Kiniksa Pharmaceuticals, Ltd., Hamilton, Bermuda
Background/Purpose: Recurrent pericarditis (RP) is an autoinflammatory disease with no FDA-approved therapies. RHAPSODY, a global Phase 3 study, evaluated rilonacept, a once-weekly IL-1α/IL-1β trap, in…
Abstract Number: 0565 • ACR Convergence 2021
Increased Risk for Inflammatory Arthritis in Veterans with Depression or Anxiety
Brian Breviu¹, Shaobo Pei¹, Matthew Kirkpatrick², Bingjian Feng¹ and Jessica Walsh³, ¹Salt Lake City Veteran Affairs and University of Utah Medical Centers, Salt Lake City, UT, ²University of Utah, Salt Lake City, UT, ³Salt Lake City Veteran Affairs Medical Center (VAMC)/University of Utah Hospital, Salt Lake City, UT
Background/Purpose: Depression and anxiety are common in patients with inflammatory arthritis (IA), and have been reported as risk factors for various inflammatory diseases. The study…
Abstract Number: 1457 • ACR Convergence 2021
Clinical Heterogeneity of the VEXAS Syndrome: A Case Series
Matthew Koster¹, Taxiarchis Kourelis¹, Kaaren Reichard¹, Tanaz Kermani², David Beck³, Daniela Ospina Cardona⁴, Matthew Samec¹, Abhishek Mangaonkar¹, Kebede Begna¹, Christopher Hook¹, Jennifer Oliveira¹, Samih Nasr¹, Benedict Tiong⁵, Mrinal Patnaik¹, Michelle Burke¹, Clement Michet¹ and Kenneth Warrington⁶, ¹Mayo Clinic, Rochester, MN, ²University of California Los Angeles, West Hills, CA, ³National Institutes of Health, Bethesda, MD, ⁴National Institutes of Health, Germantown, MD, ⁵University of California Los Angeles Medical Center, Culver City, CA, ⁶Mayo Clinic, Rochester, Minnesota
Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described myeloid-driven autoinflammatory condition caused by somatic mutations affecting methionine-41 (p.Met41) in the…
Abstract Number: 0947 • ACR Convergence 2021
Cytokine Competent Gut-joint Migratory T Cells Contribute to Inflammation in the Joint
Adam Lefferts¹, David Claypool¹, Eric Norman¹, Uma Kantheti¹ and Kristine Kuhn², ¹University of Colorado, Anschutz Medical Campus, Aurora, CO, ²University of Colorado Anschutz Medical Campus, Aurora, CO
Background/Purpose: Although studies have identified the presence of gut-associated cells in the enthesis of joints affected by spondyloarthritis, a direct link through cellular transit between…
Abstract Number: 1508 • ACR Convergence 2021
Decrease of Angiogenic T Cells Associated to the Presence of Interstitial Lung Disease in Patients with Connective Tissue Diseases
Verónica Pulito-Cueto¹, Sara Remuzgo-Martinez¹, Fernanda Genre¹, Belén Atienza-Mateo², Victor M. Mora-Cuesta³, David Iturbe-Fernández³, Leticia Lera-Gómez¹, Raquel Perez-Fernández¹, Pilar Alonso-Lecue⁴, Javier Rodriguez-Carrio⁵, Diana Prieto-Peña⁶, Virginia Portilla⁶, Ricardo BLANCO⁷, Alfosno Corrales⁶, José M. Cifrián⁸, Raquel López-Mejías¹ and Miguel Ángel gonzalez-Gay⁹, ¹Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Santander, Spain, ²Group "Research in genetic epidemiology and atherosclerosis of systemic diseases and in bone metabolic diseases of the locomotor system", IDIVAL; and Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ³Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System; Department of Pneumology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁴Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; Department of Pneumology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁵Deparment of Functional Biology, Immunology Area, Faculty of Medicine, Universidad de Oviedo, Oviedo, Asturias, Spain., Oviedo, Spain, ⁶Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁷Hospital University Marqués de Valdecilla, Santander, Spain, ⁸Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; Department of Pneumology, Hospital Universitario Marqués de Valdecilla; School of Medicine, Universidad de Cantabria, Santander, Spain, ⁹Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Division of Rheumatology, Hospital Universitario Marqués de Valdecilla; School of Medicine, Universidad de Cantabria, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa
Background/Purpose: Interstitial lung disease (ILD) is one of the most significant complications of connective tissue diseases (CTD) leading to an increase of the morbidity and…
Abstract Number: 0996 • ACR Convergence 2021
The Stimulator of Interferon Genes (STING) Protects from Bone Loss Through Regulation of Tonic and Induced Type I Interferon Pathways
Susan MacLauchlan¹, Priyanka Kushwaha¹, Albert Tai², Jia (Sijia) Chen³, Catherine Manning¹, Katherine Fitzgerald⁴, Shruti Sharma² and Ellen Gravallese⁵, ¹Brigham and Women's Hospital, Boston, MA, ²Tufts University School of Medicine, Boston, MA, ³Brigham and Women's Hospital, Cambridge, MA, ⁴University of Massachusetts Medical School, Worcester, MA, ⁵Brigham and Women's Hospital, Harvard Medical School, Chestnut Hill, MA
Background/Purpose: The intracellular DNA sensing Stimulator of Interferon Genes (STING) pathway is critical for detection of viral and bacterial pathogen DNA. Hyperactivating mutations in this…
Abstract Number: 1546 • ACR Convergence 2021
Immune Responses to COVID-19 Vaccines in Patients Using Immunosuppressive Medication for Inflammatory Arthritis – An Observational Study of 1500 Patients
Ingrid Jyssum¹, Anne Therese Tveter¹, Fridtjof Lund-Johansen², Ludvig Munthe², Sella Provan¹, Kristin Jørgensen³, Gunnveig Grødeland², Grete Kro², David Warren², Joseph Sexton¹, Tore Kvien¹, Siri Mjaaland⁴, Espen Haavardsholm¹, John Torgils Vaage², Silje Watterdal Syversen¹ and Guro Goll¹, ¹Diakonhjemmet Hospital, OSLO, Norway, ²Oslo University Hospital, Oslo, Norway, ³Akershus University Hospital, Lørenskog, Norway, ⁴Norwegian Institute of Public Health, Oslo, Norway
Background/Purpose: To assess the strength and duration of the immunological response to COVID-19 vaccines in patients treated with immunosuppressive medication for inflammatory arthritis.Methods: Adult patients…
Abstract Number: 1011 • ACR Convergence 2021
Validation of Bioinformatics Pipeline to Detect NEMO-Deleted Exon 5 Autoinflammatory Syndrome (NEMO-NDAS) and Preliminary Clinical and Immunologic Characterization
Adriana Almeida de Jesus¹, Bin Lin², Eric Karlins³, Dana Kahle⁴, Andre Rastegar², Jacob Mitchell², Sofia Torreggiani², Farzana Bhuyan², Sara Alehashemi⁵, Kader Cetin Gedik⁶, Kat Uss², Chyi-Chia Lee⁷, Hyesun Kuehn⁸, Sergio Rosenzweig⁸, Katherine Calvo⁸, Magdalena Walkiewicz⁹, Justin Lack¹⁰, Eric Hanson¹¹, Amer Khojah¹², Eveline Wu¹³, Christiaan Scott¹⁴, Timothy Ronan Leahy¹⁵, Emma MacDermott¹⁵, Orla Kileen¹⁵, Thaschawee Arkachaisri¹⁶, Zoran Gucev¹⁷, Kathryn Cook¹⁸, Vafa Mammadova¹⁹, Gulnara Nasrullayeva¹⁹, Scott Canna²⁰, Douglas Kuhns²¹, Clifton Dalgard²², Timothy Moran²³, Andrew Oler³ and Raphaela Goldbach-Mansky²⁴, ¹TADS/NIAID/NIH, Silver Spring, MD, ²TADS/NIAID/NIH, Bethesda, MD, ³BCBB/NIAID/NIH, Bethesda, MD, ⁴National Institutes of Health, Chevy Chase, MD, ⁵TADS/NIAID/NIH, Clarksville, MD, ⁶Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, ⁷NCI/NIH, Bethesda, MD, ⁸CC/DLM/NIH, Bethesda, MD, ⁹CSI/NIAID/NIH, Bethesda, MD, ¹⁰NCBR/NIAID/NIH, Bethesda, MD, ¹¹Indiana University School of Medicine, Indianapolis, IN, ¹²Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, ¹³UNC Chapel Hill, Chapel Hill, NC, ¹⁴Paediatric Rheumatology, University of Cape Town, Cape Town, South Africa, ¹⁵Children’s Health Ireland (CHI) at Crumlin, Dublin, Ireland, ¹⁶KK Women's and Children's Hospital, SingHealth, Singapore, Singapore, ¹⁷University Children's Hospital, Medical Faculty Skopje, Skopje, Macedonia, ¹⁸Akron Childrens Hospital, Copley, OH, ¹⁹Azerbaijan Medical University, Baku, Azerbaijan, ²⁰Children's Hospital of Philadelphia, Philadelphia, PA, ²¹Frederick National Laboratory for Cancer Research/NIH, Frederick, MD, ²²TAGC/USUHS, Bethesda, MD, ²³University of North Carolina School of Medicine, Chapel Hill, NC, ²⁴NIH/NIAID, Potomac, MD
Background/Purpose: Splice site variants in IKBKG that lead to exon 5 deletion cause NEMO-deleted exon 5 autoinflammatory syndrome (NEMO-NDAS). NEMO-NDAS clinically mimics the interferonopathy chronic…
Abstract Number: 1548 • ACR Convergence 2021
Q Fever as a Mimicker of Rheumatologic Conditions: A Case Series from Two Tertiary Care Academic Centers in Southern California
Manushi Aggarwal and Marven Cabling, Loma Linda University Health, Redlands, CA
Background/Purpose: Q fever, an endemic disease in Southern California, is a zoonosis caused by Coxiella burnetii. The infection can present with multiple non-specific acute and chronic manifestations including fever, headache,…
Abstract Number: 1062 • ACR Convergence 2021
Telemedicine Enriched Care Model to Optimize Care for Patients with Autoinflammatory Diseases
Lea Oefelein¹, Jens Klotsche², Susanne Benseler³, Jasmin Kuemmerle-Deschner¹ and Tatjana Welzel¹, ¹Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³University of Calgary, Calgary, AB, Canada
Background/Purpose: Autoinflammatory diseases (AID) are severe potentially life-threatening conditions requiring personalized therapy and monitoring which only few expert centers can provide. Long travel distances impede…
Abstract Number: 1618 • ACR Convergence 2021
Disease Flares in CANDLE/PRAAS with Dose Reductions of Baricitinib
Kader Cetin Gedik¹, Grace Materne², Ana Ortega-Villa³, Gina Montealegre Sanchez⁴, Adam Reinhardt⁵, Paul Brogan⁶, Yackov Berkun⁷, Sara Murias⁸, Maria Robles⁹, Susanne Schalm¹⁰, Adriana Almeida de Jesus¹¹ and Raphaela Goldbach-Mansky¹², ¹Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Diseases Section, NIAID/NIH, Bethesda, TN, ³Biostatistics Research Branch, Division of Clinical Research, NIAID/NIH, Bethesda, ⁴NIAID/NIH, Bethesda, MD, ⁵Boys Town National Research Hospital, Omaha, NE, ⁶UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁷Hadassah Hebrew University Medical Center, Jerusalem, Israel, ⁸Hospital Infantil La Paz, Madrid, Spain, ⁹Eskenazi Health Center, IndianaPolis, IN, ¹⁰Rheumatologie im Zentrum, Munich, Germany, ¹¹TADS/NIAID/NIH, Silver Spring, MD, ¹²NIH/NIAID, Potomac, MD
Background/Purpose: Patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures /proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS) respond to treatment with baricitinib but require higher exposure…
Abstract Number: 0043 • ACR Convergence 2021
Takinib Inhibits IL-1β-Induced Activation of Signal Transducer and Activator of Transcription 3 (STAT3) in Human Rheumatoid Arthritis Synovial Fibroblasts
Anil singh¹, Paul Panipinto¹, Ruby Siegel¹, Farheen Shaikh¹, Mukesh Chourasia² and Salah-uddin Ahmed¹, ¹Washington State University, Spokane, WA, ²Amity University, Noida, India
Background/Purpose: Interleukin-1 (IL-1) is a crucial mediator of inflammatory cartilage and bone destruction in rheumatoid arthritis (RA). IL-1β signaling relies on the activation of TGF-beta…
Abstract Number: 1083 • ACR Convergence 2021
Outcomes of COVID-19 Illness in Systemic Autoinflammatory Diseases and Changes in Flares During the COVID-19 Pandemic: An International Survey
Samira Nazzar Romero¹, Lakshmi Moorthy², Jennifer Tousseau³, Sivia Lapidus⁴, Mariana Correia Marques⁵, Leanne Mansfield⁶, Marinka Twilt⁷, Grant Schulert⁸, Maria Gutierrez⁹, Saskya Angevare¹⁰, Fatma Dedeoglu¹¹ and Karen Durrant¹², ¹University of California Los Angeles, Los Angeles, CA, ²Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, ³Autoinflamamtory Alliance, Lincoln, CA, ⁴The Joseph M. Sanzari Children's Hospital, Hackensack Meridian Health, Montclair, NJ, ⁵UPMC Children`s Hospital of Pittsburgh, Pittsburgh, PA, ⁶Hospital for Special Surgery, New York, NY, ⁷Alberta Children's Hospital, Calgary, AB, Canada, ⁸Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁹John's Hopkins, Baltimore, MD, ¹⁰Autoinflammatory Alliance / Kaisz / ENCA as patient organizations, Amersfoort, Netherlands, ¹¹Boston Children's Hospital, Boston, MA, ¹²Autoinflammatory Alliance, San Francisco, CA
Background/Purpose: The exaggerated inflammatory responses to the SARS-CoV-2 virus and the paucity of data on COVID-19 infection risk in systemic autoinflammatory disease (SAID) patients posed…
Abstract Number: 1632 • ACR Convergence 2021
Clinical Features and Colchicine Response in Patients with Undifferentiated Systemic Autoinflammatory Disease Carrying E148Q vs. Other MEFV Mutations
Bugra Egeli¹, Holly Wobma¹, Mariana Correia Marques², Jonathan Hausmann³ and Fatma Dedeoglu¹, ¹Boston Children's Hospital, Boston, MA, ²UPMC Children`s Hospital of Pittsburgh, Pittsburgh, PA, ³Beth Israel Deaconess Medical Center, Boston, MA
Background/Purpose: Undifferentiated systemic autoinflammatory diseases (uSAID) are diverse syndromes characterized by acute flares of fever and inflammation, which do not meet clinical criteria for known…

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