Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 1918 • ACR Convergence 2023
Nationwide Analysis of Predictors of Sarcoid Inpatient Mortality
Michael Manansala¹, Faria Sami², Shilpa Arora³ and Augustine Manadan¹, ¹Rush University Medical Center, Chicago, IL, ²John H. Stroger Jr. Hospital of Cook County, Chicago, IL, ³Cook County Hospital, Chicago, IL
Background/Purpose: Sarcoidosis is multisystem autoimmune disease characterized by noncaseating granulomas which can result in significant morbidity and mortality. This study aims to identify variables associated…
Abstract Number: 2505 • ACR Convergence 2023
Discovery of First-in-Class IRAK4 Scaffolding Inhibitors for the Treatment of Inflammatory Disorders
Kate Byth¹, Rodney Morgan², Pierre Michelys³, Kellie Demock², Geetha Kannan², Qing Chen¹, Brian Sanchez², Xiaokang Lu², Alissa Telling², Chuck Lesch², Carmen Yu², Sarah Bradley², Brian Andresen¹, Sherif Hassanien², Clarke Taylor², Zhilin Hou², Ken Borrelli¹, Anna Gardberg¹, Erika Munschy¹, Adam Schwaid¹, Anthony Opipari², Shifeng Pan³ and Luigi Franchi², ¹Odyssey Therapeutics, Boston, MA, ²Odyssey Therapeutics, Ann Arbor, MI, ³Odyssey Therapeutics, San Diego, CA
Background/Purpose: Interleukin-1 receptor-associated kinase 4 (IRAK4), is a key node that mediates inflammatory signaling in response to activation of toll-like receptor (TLR) and IL-1 receptor…
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…
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: 0039 • ACR Convergence 2022
Type I and Type II Interferon and NF-κB-Response Gene Scores and Serum Cytokine Levels in Patients with Various Systemic Autoinflammatory Diseases
Sophia Park¹, Andre Rastegar², Farzana Bhuyan³, Sara Alehashemi⁴, Danielle Fink², Douglas Kuhns², Adriana Almeida de Jesus⁵ and Raphaela Goldbach-Mansky⁶, ¹NIAID, Bethesda, MD, ²NIH, Bethesda, MD, ³National Institutes of Health, Bethesda, MD, ⁴NIH/NIAID/TADS, Clarksville, MD, ⁵NIAID, NIH, Silver Spring, MD, ⁶NIH/NIAID, Potomac, MD
Background/Purpose: Patients with systemic autoinflammatory diseases (SAIDs) present with chronic and acute elevations of proinflammatory serum cytokine and chemokine levels during exacerbations of disease flares.…
Abstract Number: 0789 • ACR Convergence 2022
The Persistence of Anti-Spike Antibodies Following Two SARS-CoV-2 Vaccine Doses in Patients with Immune-mediated Inflammatory Diseases on Immunosuppressive Therapy Compared to Healthy Controls: A Prospective Cohort Study
Ingrid Egeland Christensen¹, Ingrid Jyssum¹, Anne Therese Tveter¹, Joe Sexton¹, Siri Mjaaland², Grete Birkeland Kro³, Tore K. Kvien¹, David Warren³, Jørgen Jahnsen⁴, Ludvig A. Munthe³, Espen Haavardsholm¹, Gunnveig Grødeland³, Kristin Kaasen Jørgensen⁴, Silje Watterdal Syversen¹, Guro Løvik Goll¹ and Sella Aarrestad Provan¹, ¹Diakonhjemmet Hospital, Oslo, Norway, ²Norwegian Institute of Public Health, Oslo, Norway, ³Oslo University Hospital, Oslo, Norway, ⁴Akershus University Hospital, Oslo, Norway
Background/Purpose: The durability of vaccine-induced humoral immunity against SARS-CoV-2 in patients with immune-mediated inflammatory diseases (IMIDs) on immunosuppressive therapy is not well characterized. The aim…
Abstract Number: 1689 • ACR Convergence 2022
Oral Ginger Supplementation Counteracts NETosis in Autoimmune Mouse Models and in Healthy Humans
Ramadan Ali¹, Miela Zahavi², Christine Rysenga¹, Cyrus Sarosh³, Claire Hoy¹, Kristen Demoruelle⁴ and Jason S Knight⁵, ¹University of Michigan, Ann Arbor, MI, ²Universtiy of Michigan, Ann Arbor, MI, ³University of Michigan, Temperance, MI, ⁴University of Colorado Anschutz Medical Campus, Aurora, CO, ⁵University of Michigan, Division of Rheumatology, Ann Arbor, MI
Background/Purpose: It has previously been reported that 6-gingerol, the most abundant phytochemical in ginger root, inhibits neutrophil phosphodiesterase activity and thereby counteracts neutrophil hyperactivity in…
Abstract Number: 1943 • ACR Convergence 2022
Prevalence and Evolution of Undifferentiated Periodic Fever Syndromes in an Academic Pediatric Rheumatology Practice
Jacob Anderson¹, Catherine Bingham², Lisabeth Scalzi³, Natalya Gaffney⁴, Kimberly Hays⁵, Brandt Groh² and Timothy Hahn², ¹Phoenix Children's Hospital, Phoenix, AZ, ²Penn State Children's Hospital, Hershey, PA, ³Penn State Health, Hummelstown, PA, ⁴Self-Employed, Carlisle, PA, ⁵Penn State Health, Hershey, PA
Background/Purpose: Periodic Fever Syndromes (PFS) are common referrals to pediatric rheumatology clinics. While the syndrome of Periodic Fevers, Aphthous stomatitis, Pharyngitis, and Adenitis (PFAPA) is…

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