Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 1590 • ACR Convergence 2023
Autoimmune Pathway Blockade by a Potent Orally Bioavailable STING Antagonist
Min Yang¹, Hailong Li¹, yangyang liu¹, lili yao¹, Jing Lin¹, Zhi xie² and Wenge zhong¹, ¹Regor Therapeutics, Shanghai, China, ²Regor Pharmaceuticals, Inc., San Francisco, CA
Background/Purpose: The cyclic GMP-AMP synthase (cGAS)-stimulator of interferon genes (STING) pathway has emerged as a key innate immune mediator of autoimmune and inflammatory diseases. However,…
Abstract Number: 2044 • ACR Convergence 2023
Monogenic Interferon Mediated Diseases: Novel Phenotype and Genotype Characteristics from Saudi Population
Alhanouf Alsaleem, Sulaiman Al-Mayouf and Shahad Alansari, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia
Background/Purpose: IFN-mediated diseases are mendelian innate immunodysregulatory disorders that present early in life with fevers, sterile organ inflammation, and a high type-I IFN-response gene signature…
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…
Abstract Number: 059 • 2023 Pediatric Rheumatology Symposium
Update of Clinical and Laboratory Features of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease (SJIA-LD) Cohort
Esraa Eloseily¹, Min-Lee Chang², Mary Ellen Riordan³, allan Russell⁴, Marc Natter², Yukiko Kimura⁵ and Grant Schulert¹, ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Boston Children's Hospital, Boston, MA, ³Hackensack Meridian Health/ Joseph M. Sanzari Children's Hospital, Hackensack, NJ, ⁴Duke Clinical Research Institute, Durham, NC, ⁵Hackensack Meridian School of Medicine, Hackensack, NJ
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) associated lung disease (SJIA-LD) is an emerging and life-threatening clinical problem. Despite recent advances, there remain key unanswered questions…
Abstract Number: 068 • 2023 Pediatric Rheumatology Symposium
Outcomes of Children with Uveitis Associated with Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy (ADNIV)
Ilaria Maccora¹, Arjun Sood², Grant Schulert³, Megan Quilan-Water³, Alexandra Duell³, Jennifer Huggins³, Tiffany Nguyen², Cameron Sapp², Sumit Sharma⁴, Srivastaval Sunil⁴ and Sheila Angeles-Han⁵, ¹IRCCS Meyer Children’s Hospital, Rheumatology Unit, Florence, Italy, ²Cincinnati Eye Institute, Cincinnati, OH, ³Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Cole Eye Institute, Cleveland Clinic, Cleveland, OH, ⁵Cincinnati Children's Hospital, Cincinnati, OH
Background/Purpose: Pediatric uveitis is commonly associated with rheumatic disease and can lead to sight-threatening complications if not properly treated. Systemic immunomodulatory therapy has dramatically changed…
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: 0166 • ACR Convergence 2022
Detection of the Novel Autoantibodies Against Transcription Factor Sp4 Is Related with Low Risk of Cancer in Idiopathic Inflammatory Myopathy Patients
Yuji Hosono¹, Azusa Kojima¹, Akira Ishii¹, Mai Sugiyama¹, Yuto Izumi¹, Noriko Sasaki², Chiho Yamada¹ and Shinji Sato¹, ¹Tokai University School of Medicine, Isehara Kanagawa, Japan, ²Tokai University School of Medicine, sagamihara-city, Japan
Background/Purpose: In idiopathic inflammatory myopathy (IIM), many kinds of autoantibodies are often detected and associated with each clinical phenotype. Recently, autoantibody against transcription factor Sp4…
Abstract Number: 1107 • ACR Convergence 2022
Efficacy and Safety of Tofacitinib in Patients with Polymyalgia Rheumatica (EAST PMR): A Prospective Study
Xinlei Ma, Fan Yang, Jin Lin and Weiqian Chen, Division of Rheumatology, the First Affiliated Hospital, Zhejiang University School of Medicine, HangZhou, China
Background/Purpose: Polymyalgia Rheumatica (PMR) is a common inflammatory disease in elderly persons whose pathogenesis is unclear. Glucocorticoids are the first-line drugs for patients with PMR,…
Abstract Number: 1842 • ACR Convergence 2022
Vaccination in Patients with Autoinflammatory Periodic Syndromes Under Canakinumab – Safety Data Interim Analysis of the RELIANCE Registry
Jasmin B. Kuemmerle-Deschner¹, Joerg Henes², Birgit Kortus-Goetze³, Tilmann Kallinich⁴, Prasad T. Oommen⁵, Juergen Rech⁶, Tobias Krickau⁷, Frank Weller-Heinemann⁸, Gerd Horneff⁹, Ales Janda¹⁰, Ivan Foeldvari¹¹, Catharina Schuetz¹², Frank Dressler¹³, Michael Borte¹⁴, Markus Hufnagel¹⁵, Florian Meier¹⁶, Michael Fiene¹⁷, Julia Weber-Arden¹⁸ and Norbert Blank¹⁹, ¹Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, Germany, ²Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, ³Division of Nephrology, University of Marburg, Marburg, Germany, ⁴Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ⁵Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁶University Clinic Erlangen, Erlangen, Germany, ⁷Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Pediatrics, Erlangen, Germany, ⁸Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ⁹Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ¹⁰Department of Pediatrics, University Hospital Ulm, Ulm, Germany, ¹¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ¹²Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, ¹³Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, ¹⁴ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Germany, Leipzig, Sachsen, Germany, ¹⁵Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, ¹⁶Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany, and Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt, Germany, ¹⁷Rheumatology Center Greifswald, Greifswald, Germany, ¹⁸Novartis Pharma GmbH, Nuernberg, Germany, ¹⁹Rheumatology, University Hospital Heidelberg, Heidelberg, Germany
Background/Purpose: Treatment of autoinflammatory periodic syndromes with the interleukin-1β inhibitor canakinumab (CAN) has been shown to be safe and effective in clinical trials and in…
Abstract Number: 0548 • ACR Convergence 2022
Postural Orthostatic Tachycardia Syndrome, Small Fiber Neuropathy, and Systemic Inflammation: Are They Related?
Elizabeth Bettini, Carolyn Ramwell, Sangeeta Sule and Jeffery Moak, Children's National Hospital, Washington, DC
Background/Purpose: Postural Orthostatic Tachycardia Syndrome (POTS) is a dysautonomia associated with chronic autonomic and neuropathic symptoms, including pain in multiple body regions, paresthesia, fatigue, brain…

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