Abstracts tagged "Autoinflammatory diseases"

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…
Abstract Number: 0045 • ACR Convergence 2021
Endothelial Cells from Patients with DMARD Naïve, Active Inflammatory Arthritis Demonstrate Pro-inflammatory Sensitisation That Is Reversed by Therapy Initiation
Robert Maughan¹, Marie Lang¹, Anton Olsson¹, Andrew Porter¹, Antonia Greeves², Taryn Youngstein³, Charis Pericleous¹ and Justin Mason¹, ¹Imperial College London, London, United Kingdom, ²Hammersmith Hospital, London, United Kingdom, ³Hammersmith Hospital & Imperial College London, London, United Kingdom
Background/Purpose: Inflammatory arthritides (IA) including rheumatoid arthritis are associated with increased cardiovascular disease (CVD) mortality. Although inflammation-driven endothelial dysfunction is considered a major factor, the…
Abstract Number: 1104 • ACR Convergence 2021
Pathogenic UBA1 Variants in Japanese Patients with Relapsing Polychondritis
Naomi Tsuchida¹, Yosuke Kunishita², Yuri Uchiyama¹, Yohei Kirino³, Kaoru Takase-Minegishi¹, Ryusuke Yoshimi¹, Hideaki Nakajima¹ and Naomichi Matsumoto¹, ¹Yokohama City University Graduate School of Medicine, Yokohama, Japan, ²Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan, ³Yokohama City University, Yokohama, Japan
Background/Purpose: Pathogenic somatic variants in the ubiquitin like modifier activating enzyme 1 gene (UBA1) were discovered in individuals with systemic inflammation of cartilage, skin, and…
Abstract Number: 1707 • ACR Convergence 2021
A Bioengineered Probiotic for the Oral Delivery of an Immunomodulator in Rheumatoid Arthritis
Yuqing Wang¹, Duolong Zhu¹, Laura Ortiz-Velez², Jacob Perry¹, Michael Pennington³, Joseph Hyser¹, Robert Britton¹ and Christine Beeton¹, ¹Baylor College of Medicine, Houston, TX, ²TMC Innovation, Houton, ³AmbioPharm Inc., North Augusta, SC
Background/Purpose: CCR7- effector memory T (TEM) lymphocytes are targets for immunomodulation for the treatment of rheumatoid arthritis (RA). Following activation, TEM cells upregulate the expression…
Abstract Number: 0162 • ACR Convergence 2021
Cardiac Magnetic Resonance Imaging for Guiding Decision-making on Treatment Duration: Data from RHAPSODY, a Phase 3 Clinical Trial of Rilonacept in Recurrent Pericarditis
Paul Cremer¹, David Lin², Alistair Wheeler³, Antonio Abbate⁴, Antonio Brucato⁵, Fang Fang⁶, Antonella Insalaco⁷, Martin LeWinter⁸, Basil S. Lewis⁹, Sushil A. Luis¹⁰, Stephen J. Nicholls¹¹, John Petersen¹², Allan Klein¹, Massimo Imazio¹³ and John F Paolini⁶, ¹Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, ²Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, ³Kiniksa Pharmaceuticals, Ltd., Hamilton, Bermuda, ⁴VCU Pauley Heart Center, Virginia Commonwealth University, Richmond, VA, ⁵Department of Biomedical and Clinical Science, University of Milano, Fatebenefratelli Hospital, Milan, Italy, ⁶Kiniksa Pharmaceuticals Corp., Lexington, MA, ⁷Department of Pediatric Medicine, Division of Rheumatology, Bambino Gesù Children's Hospital, Rome, Italy, ⁸Cardiology Unit, The University of Vermont Medical Center, The University of Vermont, Burlington, VT, ⁹Cardiovascular Clinical Research Institute, Lady Davis Carmel Medical Center, Haifa, Israel, ¹⁰Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, NY, ¹¹MonashHeart, Department of Cardiology, Monash University, Clayton, Australia, ¹²Swedish Medical Center, Seattle, WA, ¹³University Cardiology, Cardiovascular and Thoracic Department, AOU Città della Salute e della Scienza di Torino, Torino, Italy
Background/Purpose: The magnitude of pericardial delayed hyperenhancement (DHE) by cardiac magnetic resonance imaging (CMR) illustrates the severity of inflammation in pericarditis. We hypothesized that patients…
Abstract Number: 1105 • ACR Convergence 2021
Discovering Variants in Suspected Monogenic Systemic Inflammatory Disease: An Adult Case Series
Jason An¹, Madeline Couse¹, Dilan Dissanayake², Daniela Dominguez², Ronald Laxer¹, Christian Marshall¹, Johannes Roth³, Robert Rottapel⁴ and Linda Hiraki², ¹SickKids, Toronto, ON, Canada, ²The Hospital for Sick Children, Toronto, ON, Canada, ³University of Ottawa, Ottawa, ON, Canada, ⁴St Michael's Hospital, Toronto, ON, Canada
Background/Purpose: Monogenic systemic inflammatory diseases (MSID) are a heterogeneous group of rare conditions caused by single gene variants leading to immune dysregulation. Diagnostic yield of…
Abstract Number: 1769 • ACR Convergence 2021
BMS-986256, an Oral Novel Toll-like Receptor 7 and 8 (TLR7/8) Inhibitor, Does Not Affect the Pharmacokinetics of Mycophenolate Mofetil in Healthy Subjects
Manoj Chiney¹, Ihab Girgis², Melanie Harrison¹, Xiaoping Zhang¹, Yun Shen³, Michelle Dawes¹, Lixian Dong², Diane Shevell³, Urvi Aras¹ and Bindu Murthy³, ¹Bristol Myers Squibb, Lawrenceville, NJ, ²Bristol Myers Squibb, Princeton, NJ, ³Bristol Myers Squibb, Lawrenceville
Background/Purpose: Toll like receptor (TLR) 7 and TLR8 are activated as part of the disease pathophysiology of systemic lupus erythematosus (SLE), including lupus nephritis (LN),…
Abstract Number: 0189 • ACR Convergence 2021
Tapering and Discontinuation of Background Therapies During the Transition to Rilonacept Monotherapy in RHAPSODY, a Phase 3 Clinical Trial of Rilonacept in Patients with Recurrent Pericarditis
Antonio Brucato¹, Alistair Wheeler², Sushil A. Luis³, Antonio Abbate⁴, Paul Cremer⁵, Fang Fang⁶, Antonella Insalaco⁷, Martin LeWinter⁸, Basil S. Lewis⁹, David Lin¹⁰, Stephen J. Nicholls¹¹, Allan Klein⁵, Massimo Imazio¹² and John F Paolini⁶, ¹Department of Biomedical and Clinical Science, University of Milano, Fatebenefratelli Hospital, Milan, Italy, ²Kiniksa Pharmaceuticals, Ltd., Hamilton, Bermuda, ³Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, NY, ⁴VCU Pauley Heart Center, Virginia Commonwealth University, Richmond, VA, ⁵Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, ⁶Kiniksa Pharmaceuticals Corp., Lexington, MA, ⁷Department of Pediatric Medicine, Division of Rheumatology, Bambino Gesù Children's Hospital, Rome, Italy, ⁸Cardiology Unit, The University of Vermont Medical Center, The University of Vermont, Burlington, VT, ⁹Cardiovascular Clinical Research Institute, Lady Davis Carmel Medical Center, Haifa, Israel, ¹⁰Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, ¹¹MonashHeart, Department of Cardiology, Monash University, Clayton, Australia, ¹²University Cardiology, Cardiovascular and Thoracic Department, AOU Città della Salute e della Scienza di Torino, Torino, Italy
Background/Purpose: Post-episode tapering of Standard of Care (SoC) medication in patients with recurrent pericarditis (RP) varies considerably. Gradual tapering of corticosteroids (CS) is recommended in…

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