Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 0220 • ACR Convergence 2023
Paxlovid (Nirmatrelvir Plus Ritonavir) in Patients with Underlying Rheumatological Diseases, in Preventing COVID-19 Related Hospitalization and Death
Faiza Javed, Tony Mangino and Paramarajan Piranavan, University of Kentucky College of Medicine, Lexington, KY
Background/Purpose: The benefits of antiviral therapy in the COVID-19 patients with underlying rheumatic disease with or without immunosuppression are not entirely clear. The goal of…
Abstract Number: 0891 • ACR Convergence 2023
Selective and Potent Inhibition of Cyclic GMP-AMP Synthase (cGAS) Fully Normalizes Autoinflammation Across Tissues in a Trex1-/- Mouse Model for Type I Interferonopathies
Arnaud Bourin¹, Zuzana Hořejší², Isabelle Cambré¹, Juraj Dobiaš², Mikhail Klychnikov², Radek Liboska², Maroš Smolíček², Zdeněk Vavřina², Wanda Haeck¹, Hugo Klaassen¹, Marnik Nijs¹, Kristine Metzger¹, Sandro Boland¹, Dries De Clercq¹, Sara Allasia¹, Gunter Carlens¹, Patrick Chaltin³, Arnaud Marchand¹, Ondřej Páv², Matthias Versele¹ and Gabriel Birkuš², ¹Centre for Drug Design and Discovery (CD3) - Cistim, Leuven, Belgium, ²Institute of Organic Chemistry and Biochemistry, Prague, Czech Republic, ³Centre for Drug Design and Discovery (CD3) - KU Leuven, Leuven, Belgium
Background/Purpose: The cGAS enzyme is a sensor of cytosolic double-strand (ds) DNA. It serves to detect viruses and elicits an acute, transient cGAMP-STING mediated type…
Abstract Number: 1775 • ACR Convergence 2023
The Peptidyl Arginine Deiminase Inhibitor BB-CLA Decreases the Inflammatory and Fibrotic Responses in Macrophages and Rheumatoid Arthritis Synovial Fibroblasts Exposed to Fibrinogen Modified with Malondialdehyde-Acetaldehyde Adduct and Citrulline
Jack Mordeson¹, Nozima Aripova¹, Michael Duryee¹, James O'Dell¹, Bryant England¹, Daniel Anderson¹, Ted R Mikuls² and Geoffrey Thiele¹, ¹University of Nebraska Medical Center, Omaha, NE, ²Division of Rheumatology and Immunology, University of Nebraska Medical Center, Omaha, NE
Background/Purpose: Peptide citrullination and adduction with malondialdehyde-acetaldehyde adduct (MAA) are post-translational modifications involved in the pathogenesis of rheumatoid arthritis (RA). Anti-cyclic-citrullinated peptide antibodies are >90%…
Abstract Number: 2053 • ACR Convergence 2023
Predictive Factors Associated with Treatment Response in Chronic Nonbacterial Osteomyelitis
Katherine Nowicki¹, Nathan Rogers², Carson Keeter², Nathan Donaldson², Jennifer Soep² and Yongdong (Dan) Zhao³, ¹University of Colorado, Lakewood, CO, ²University of Colorado, Aurora, CO, ³University of Washington, Redmond, WA
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is characterized by sterile inflammatory bone lesions and most commonly affects skeletally immature children. Non-steroidal anti-inflammatory drugs (NSAIDs) are the…
Abstract Number: 0255 • ACR Convergence 2023
Lymphoid Mass in the Inferior Turbinate with IgG4 Producing Cells: A Nasal Manifestation of IgG4-RD or a Distinct Chronic Inflammatory Disease?
Ji In Jung¹, Ju Yeon Kim², Jun Won Park², Eun Bong Lee², Jin Kyun Park³ and Doo Hee Han², ¹Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, South Korea, ²Seoul National University Hospital, Seoul, South Korea, ³Seoul National University College of Medicine, Seoul, South Korea
Background/Purpose: IgG4-related disease (IgG4-RD) is a rare, multi-system, fibro-inflammatory disorder characterized by the infiltration of lymphoplasmacytic cells, storiform fibrosis, obliterative phlebitis, and the presence of…
Abstract Number: 1093 • ACR Convergence 2023
Development of Adaptive Immunity Against Different SARS-CoV-2 Variants over the Course of Three COVID-19 Vaccinations in Patients with Inflammatory Rheumatic Diseases
Liam Huppke¹, Paul Wratil², Marie Bischof², Stefan Wolfrum¹, Fabian Ullrich¹, Delila Singh¹, Julia Lichtnekert¹, Lea Grümme¹, Christina Gebhardt¹, Klaus Krüger³, Franziska Wiesent⁴, Alla Skapenko¹, Oliver Keppler⁵ and Hendrik Schulze-Koops⁶, ¹Division of Rheumatology and Clinical Immunology, Department of Internal Medicine IV, University of Munich, Munich, Germany, ²Max von Pettenkofer Institute & Gene Centre, Virology, National Reference Centre for Retroviruses, LMU Munich, Munich, Germany, ³Praxiszentrum St. Bonifatius, Munich, Germany, ⁴Endokrinologikum München, Munich, Germany, ⁵Max von Pettenkofer Institute & Gene Centre, Virology, National Reference Centre for Retroviruses, LMU München, Munich, Germany, ⁶Division of Rheumatology and Clinical Immunology, Department of Internal Medicine IV, Ludwig-Maximilians–University Munich, Munich, Germany
Background/Purpose: Even though individuals with inflammatory rheumatic diseases (IRDs) were excluded from the SARS-CoV-2 vaccine trails, studies have shown that these individuals at risk of…
Abstract Number: 1817 • ACR Convergence 2023
Geographic Variability of Inflammatory Bowel Disease Related Mortality, 1999-2019; CDC WONDER
Saleha Aziz¹, Anum Akhlaq¹, Sana Gurz² and Yousaf Zafar¹, ¹University of Mississippi Medical Center, Jackson, MS, ²University of Nevada, Las Vegas, NV
Background/Purpose: The United States makes a significant contribution to Inflammatory Bowel Disease (IBD) related disease burden worldwide. However, data on geographic variability of IBD related…
Abstract Number: 2058 • ACR Convergence 2023
IL-1 Blocking Treatment Slows the Progression of Sensorineural Hearing Loss in Patients with NOMID
Sara Alehashemi¹, Ana M. Ortega-Villa², Megha Garg³, Katherine Myint-Hpu⁴, Kim Johnson⁵, FARZANA BHUYAN⁶, Kelly King⁷, Chris Zalewski⁷, Danielle Fink⁸, Douglas B. Kuhns⁸, John Butman⁹, Carmen Brewer⁷, H. Jeffrey Kim⁷, Dean Follmann² and Raphaela Goldbach-Mansky¹⁰, ¹NIH/NIAID/TADS, Clarksville, MD, ²Biostatistics Research Branch, Division of Clinical Research, NIH, Bethesda, MD, ³Rochester Regional Health, Pittsford, NY, ⁴NIAID, NIH, Bethesda, MD, ⁵NIH, NIAID, Bethesda, MD, ⁶National Institutes of Health, Bethesda, MD, ⁷National Institute on Deafness and Other Communication Disorders, NIH, Bethesda, MD, ⁸Frederick National Laboratory for Cancer Research, Bethesda, MD, ⁹Clinical Center, NIH, Bethesda, MD, ¹⁰NIH/NIAID, Potomac, MD
Background/Purpose: NOMID is a severe form of cryopyrin-associated periodic syndrome characterized by systemic inflammation and CNS manifestations, including sensorineural hearing loss. IL-1 blocking agents, Anakinra…
Abstract Number: 077 • 2023 Pediatric Rheumatology Symposium
Clinical Characteristics of Chronic Recurrent Multifocal Osteomyelitis or Chronic Nonbacterial Osteomyelitis in Pediatrics; Single Center Study in Korea
Minji Kim¹, Jieun Jeong¹, Jiwon Jung², Seong Heon Kim¹ and Seon Hee Lim³, ¹Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Republic of Korea, ²Asan Medical Center Children's Hospital, Seoul, Republic of Korea, ³Department of Pediatrics, Pusan National University Children’s Hospital, Yangsan, Republic of Korea
Background/Purpose: CRMO is an autoinflammatory bone disease which has a recurrent bone pain as a symptom. It causes problems in various organs, including joints, skin,…
Abstract Number: 078 • 2023 Pediatric Rheumatology Symposium
Periodic Fever Syndrome and Myelodysplastic Syndrome: Possible Connections Between Two Disorders
Ivanna Romankevych¹, Nicole Torres², Imani Sanders¹, Laurent King¹ and Pedro Garcia Rodriguez¹, ¹Jackson Health System, University of Miami Miller School of Medicine, Miami, FL, ²University of Miami Miller School of Medicine, Miami, FL
Background/Purpose: Background: associations between autoimmune, immunodeficiency and hematological disorders are well known as well as with autoinflammatory conditions. Modern genetic testing allows to link these…
Abstract Number: 121 • 2023 Pediatric Rheumatology Symposium
The Effectiveness of Tonsillectomy in Periodic Fever, Aphthous Ulcer, Pharyngitis, and Adenitis Syndrome in Pediatric Patients
John Storwick¹ and Marinka Twilt², ¹Alberta Children's Hospital/University of Calgary, Calgary, AB, Canada, ²Alberta Children's Hospital, Calgary, AB, Canada
Background/Purpose: Periodic Fevers with Aphthous Ulcers, Pharyngitis, and Adenitis Syndrome (PFAPA Syndrome) is the most common pediatric periodic fever syndrome. The most recent diagnostic criteria…
Abstract Number: 127 • 2023 Pediatric Rheumatology Symposium
What’s in a Name? A20 Protein Expression in an in Vitro Model of A20 Haploinsufficiency
Patricia Pontes Aires¹, DANIELA GERENT PETRY PIOTTO¹, Andre Cunha¹, Sandro Perazzio² and Maria Teresa Terreri³, ¹Universidade Federal de São Paulo, São Paulo, Brazil, ²Universidade de So Paulo; Fleury Laboratories, São Paulo, Brazil, ³UNIFESP, São Paulo, Brazil
Background/Purpose: Tumor necrosis factor alpha (TNF-alpha) induced protein 3 gene, or TNFAIP3, encodes the A20 protein, an important regulator of the NF-κB pathway. Since its…
Abstract Number: 032 • 2023 Pediatric Rheumatology Symposium
From Bedside to Bench and Back: Discovery of a Novel Missense Variant in NLRP3 Causing Atypical Cryopyrin-Associated Periodic Syndromes with Hearing Loss as the Primary Presentation, Responsive to Anti-IL1 Therapy
Merav Birk-Bachar¹, Hadar Cohen², Yoel Levinsky³, rotem tal⁴, Gil Amarilyo⁵, Meirav Sokolov⁶, Efrat Sofrin-Drucker⁷, Naama Orenstein⁷, Gabriel Lidzbarsky⁷, Liora Kornreich⁸, Eyal Raveh⁶, Nesya Kropach-Gilad⁷, Motti Gerlic² and Liora Harel⁹, ¹Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ²Sackler Faculty of Medicine, Immunology Department, Tel Aviv University, Tel Aviv, Israel, ³Schneider Children's Medical Center of Israel, Tel Aviv University, Petach Tikva, Israel, ⁴Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ⁵Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ⁶Pediatric Ear Nose and Throat Unit Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ⁷Recanati Genetic Institute, Rabin Medical Center-Beilinson Hospital, Petach Tikva, Israel, ⁸Pediatric Imaging Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ⁹Scheiders Children Medical Center of Israel, Petah-Tiqva, Israel
Background/Purpose: Cryopyrin-associated periodic syndromes (CAPS) are a spectrum of rare autoinflammatory diseases caused by gain-of-function mutations inthe NLRP3 gene. These mutations cause inflammasome hyperactivity and…
Abstract Number: 036 • 2023 Pediatric Rheumatology Symposium
Predictive Factors of Long-lasting Remission Following Anakinra Withdrawal in Patients with Systemic Juvenile Idiopathic Arthritis After Achievement of Clinical Inactive Disease
Germana Nardini¹, Claudia Bracaglia², Denise Pires Marafon¹, Emanuela Sacco³, Arianna De Matteis¹, Ivan Caiello¹, Giusi Prencipe¹, Fabrizio De Benedetti² and Manuela Pardeo², ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Roma, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, ³Fondazione Casa Sollievo della Sofferenza, Pediatria, San Giovanni Rotondo, Italy
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a rare autoinflammatory disease of unknown etiology. Several uncontrolled studies showed that early treatment with anakinra is associated…
Abstract Number: 044 • 2023 Pediatric Rheumatology Symposium
Validation of Newly Proposed Classification Criteria for Pediatric Chronic Nonbacterial Osteomyelitis: A Virginia Cohort
Kelley Lee¹, Ashley Kim² and Aarat Patel³, ¹University of Richmond, Richmond, VA, ²University of Virginia, Charlottesville, VA, ³Bon Secours Mercy Health / University of Virginia, GLEN ALLEN, VA
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is an aseptic autoinflammatory bone disease of unknown etiology. This diagnosis can be delayed due to the non-specific nature of…

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