Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 0965 • ACR Convergence 2020
Repetitive Inhalant Lipopolysaccharide Exposure in the Setting of Arthritis Induction Potentiates Pro-Fibrotic Inflammatory Lung Disease in Mice
Madison Wolfe¹, Ted Mikuls¹, Geoffrey Thiele¹, Amy Nelson¹, Michael Duryee¹, Rohit Gaurav¹, Bryant England¹, Debra Romberger¹ and Jill Poole¹, ¹University of Nebraska Medical Center, Omaha, NE
Background/Purpose: Rheumatoid arthritis (RA) is associated with several lung diseases. Various airborne exposures have been implicated as RA disease risk factors including cigarette smoke and…
Abstract Number: 1479 • ACR Convergence 2020
Long-Term Effectiveness of Canakinumab in Autoinflammatory Diseases – Interim Analysis of the CAPS Subgroup from the RELIANCE Registry
Jasmin Kuemmerle-Deschner¹, Birgit Kortus-Goetze², Michael Borte³, Ivan Foeldvari⁴, Gerd Horneff⁵, Ales Janda⁶, Tilmann Kallinich⁷, Prasad T. Oommen⁸, Catharina Schuetz⁹, Frank Weller-Heinemann¹⁰, Julia Weber-Arden¹¹ and Norbert Blank¹², ¹University Hospital Tuebingen, Tuebingen, Germany, ²Division of Nephrology, University of Marburg, Germany, Marburg, Germany, ³ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Germany, Leipzig, Germany, ⁴Head of the Hamburg Centre for Pediatric and Adolescence Rheumatology, Budapest, Hungary, ⁵Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ⁶Department of Pediatrics, University Hospital Ulm, Germany, Ulm, Germany, ⁷Charite, Berlin, Germany, ⁸Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁹Department of Pediatric Immunology, University Hospital Dresden, Dresden, Germany, Dresden, Germany, ¹⁰Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, Bremen, Germany, ¹¹Novartis Pharma GmbH, Nuremberg, Germany, ¹²Department of Hematology, Oncology and Rheumatology, Internal Medicine V, University Hospital of Heidelberg, Eppelheim, Germany
Background/Purpose: In the treatment of monogenic autoinflammatory diseases (AID), a heterogeneous group of diseases with excessive interleukin (IL)-1β release and severe systemic and organ inflammation,…
Abstract Number: 0097 • ACR Convergence 2020
Does Testing for SAA Is More Beneficial Than CRP for the Follow-up of Patients with FMF?
Mert Oztas¹, Serdal Ugurlu¹, Oguzhan Selvi², Bilgesu Ergezen¹ and Huri Ozdogan¹, ¹Istanbul University-Cerrahpasa, Istanbul, Turkey, ²Okmeydani Research and Training Hospital, Istanbul, Turkey
Background/Purpose: In order to follow subclinical inflammation and adjust the therapy for an optimal disease control, clinicians seek for readily accessible, affordable and reproducible markers.…
Abstract Number: 1035 • ACR Convergence 2020
Study of Ocular Sarcoidosis and Clusters of Clinical Associations in a Series of 383 Patients with Systemic Sarcoidosis from a Single Hospital
Inigo Gonzalez-Mazon¹, Carmen Alvarez-Reguera², Lara Sanchez-Bilbao², David Martinez-Lopez³, Alba Herrero Morant², Jorge Javier Gaitan-Valdizan⁴, Raul Fernandez-Ramon², Rosalia Demetrio-Pablo² and Ricardo Blanco², ¹Hospital Universitario Marques de Valdecilla, Bezana, Spain, ²Hospital Universitario Marques de Valdecilla, Santander, Spain, ³Hospital Universitario Marques de Valdecilla, Santander (SPAIN), Spain, ⁴Hospital Universitario Marqués de Valdecilla, Santander, Spain
Background/Purpose: Sarcoidosis is an inflammatory disease which can affect multiple organs. The most frequent affected organs are lungs, skin and eyes. Ocular involvement is a…
Abstract Number: 1531 • ACR Convergence 2020
Characterization of Cytokine/chemokine Profile in Patient-derived M1/ M2 Macrophages to Identify Biomarkers for Genetically-defined Systemic Autoinflammatory Diseases
Farzana Bhuyan¹, Adriana Almeida de Jesus², Kim Johnson³, Jacob Mitchell⁴, Yan Huang⁵ and Raphaela Goldbach-Mansky⁵, ¹NIH, bhetesda, MD, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, ³NIH, NIAID, Bethesda, ⁴Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ⁵NIH, Bethesda
Background/Purpose: Genetic mutations in key regulatory molecules of the innate immune system cause autoinflammatory diseases through propagation of hyperinflammatory responses. Monocytes/ macrophages regulate inflammatory processes…
Abstract Number: 0161 • ACR Convergence 2020
Validation of Patient-reported Outcomes (PRO) Lung Questionnaires for Systemic Juvenile Idiopathic Arthritis (SJIA) Patients at Risk for Lung Disease
Kim Nguyen¹, Christopher Towe², Shima Yasin³, Alexei Grom⁴, Hermine I Brunner⁵ and Grant Schulert⁵, ¹Veterans Affairs Cincinnati, University of Cincinnati Medical Center, Cincinnati, ²Cincinnati Children's Medical Hospital Center, Cincinnati, ³Cincinnati Children's Hospital Medical Center, Cincinnati, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) involves dysregulation of inflammation and innate immunity, and can cause life-threatening complications including lung disease (LD). However, there are…
Abstract Number: 1063 • ACR Convergence 2020
Spirulina Stimulates Inflammatory Cytokine Production Through the STING and TLR Pathways in Dermatomyositis in Vitro
Christina Bax¹, Yubin Li¹, Spandana Maddukuri², Adarsh Ravishankar³, Jay Patel³, Daisy Yan¹, Josef Symon Concha¹ and Victoria Werth¹, ¹University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia, PA, ²University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Montville, NJ, ³University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia
Background/Purpose: Spirulina, a popular herbal supplement, stimulates the immune system, as determined by in vitro and in vivo studies. Our recent epidemiologic data suggest that Spirulina…
Abstract Number: 1578 • ACR Convergence 2020
Rituximab Hypersensitivity in Rheumatic and Inflammatory Diseases: Role of Skin Testing
Sravani Penumarty¹, Javier Quintero Betancourt², Eugenio Capitle² and Reena Khianey², ¹Rutgers, Eastvale, CA, ²Rutgers, Newark, NJ
Background/Purpose: Infusion-related reactions have been reported with rituximab, a monoclonal antibody targeting the CD20 antigen on B cells, and may result in discontinuation of the…
Abstract Number: 0162 • ACR Convergence 2020
Identifying Sleep Problems in Systemic Juvenile Idiopathic Arthritis (SJIA) Patients with Patient-reported Outcomes (PRO) Questionnaires
Kim Nguyen¹, Christopher Towe², Shima Yasin³, Alexei Grom⁴, Hermine I Brunner⁵ and Grant Schulert⁵, ¹Veterans Affairs Cincinnati, University of Cincinnati Medical Center, Cincinnati, ²Cincinnati Children's Medical Hospital Center, Cincinnati, ³Cincinnati Children's Hospital Medical Center, Cincinnati, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) can cause severe and chronic multisystem involvement. Medical therapies including high-dose corticosteroids can have significant side effects affecting sleep.…
Abstract Number: 1152 • ACR Convergence 2020
IL-18: A Biomarker That Reflects Disease Activity, Could It Be the Next Disease Activity Measure in Systemic Juvenile Idiopathic Arthritis?
Shima Yasin¹, Thuy Do², Sanjeev Dhakal², Elizabeth Baker², Alexei Grom³ and Grant Schulert⁴, ¹Cincinnati Children's Hospital Medical Center, Liberty twp, OH, ²Cincinnati Children's Hospital Medical Center, Cincinnati, ³Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a childhood arthritis with prominent innate immune activity. Disease presentation and flares could largely mimic infections with fever…
Abstract Number: 1636 • ACR Convergence 2020
8 Years Follow-Up of a Novel Autoinflammatory Disease: CD59 Malfunction Causes Hemolytic Anemia, Recurrent Guillain-Barre Syndrome, and Strokes in Pediatric Populations and Respond Well to Eculizumab and Pozelimab
Dror Mevorach¹ and Netanel Karbian¹, ¹Hadassah-University Hospital, Jerusalem, Yerushalayim, Israel
Background/Purpose: In 2013 we have described the first patients with a novel autoinflammatory disease manifested in 4 children with recurrent Guillain-Barre syndrome and hemolytic anemia…
Abstract Number: 0164 • ACR Convergence 2020
What’s in a Name? Patient and Family Perspectives on the Naming of Systemic Juvenile Idiopathic Arthritis
Mariana Correia Marques¹, Rashmi Sinha², Karen Durrant³, Sivia Lapidus⁴, Nicole Tennermann⁵, Saskya Angevare⁶, Leah Bush⁷, Kari Cupp⁸, Jonathan Hausmann⁹, David Maher¹⁰, Shalla Newton¹⁰, Michael Ombrello¹¹, Phillip Reardon⁸, Rebecca Trachtman¹², Fatma Dedeoglu⁵ and Grant Schulert¹³, ¹Boston Children`s Hospital, Department of Pediatrics, Harvard Medical School, Boston, MA, ²SJIA Foundation, Cincinnati, ³Autoinflammatory Alliance, San Francisco, CA, ⁴The Joseph M. Sanzari Children's Hospital, Hackensack Meridian Health, Montclair, NJ, ⁵Boston Children's Hospital, Boston, MA, ⁶Autoinflammatory Alliance, Amersfoort, Netherlands, ⁷Systemic JIA Foundation, Cincinnati, OH, ⁸Systemic JIA Foundation, Cincinnati, ⁹Boston Children's Hospital / Beth Israel Deaconess Medical Center, Cambridge, MA, ¹⁰Still's Disease, the 411, National organization, ¹¹Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD, ¹²Icahn School of Medicine at Mount Sinai, New York, NY, ¹³PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: The childhood inflammatory disorder systemic juvenile idiopathic arthritis (SJIA) has historically had several names, including Still’s disease and systemic juvenile rheumatoid arthritis. While its…
Abstract Number: 1156 • ACR Convergence 2020
Comparison of Immunological Biomarkers and Lung Histology in Patients with Elevated IL18 – Pulmonary Alveolar Proteinosis and Recurrent Macrophage Activation Syndrome (IL-18PAP-MAS) and Other Inflammatory Lung Diseases
Alhanouf Alsaleem¹, Adriana de Jesus², Sofia Torreggiani³, Chyi-Chia Lee⁴, Les Folio⁵, Huy Do⁶, Andrew Oler⁷, Caroline Kim³, Stewart Levine⁸, Anthony Suffredini⁹, Cem Gabay¹⁰, Joseph Fontana¹¹, Scott Canna¹² and Raphaela Goldbach-Mansky¹³, ¹Division of Pediatric Rheumatology, Department of pediatrics, King Faisal specialist hospital and research center, Riyadh, Saudi Arabia, RiYADH, Saudi Arabia, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, ³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ⁴Pathology Department/NCI/NIH, Bethesda, MD, ⁵Radiology and Imaging Services/NIH, Bethesda, MD, ⁶Radiology and Imaging Sciences, Bethesda, MD, ⁷Bioinformatics and Computational Biosciences Branch/NIAID/NIH, Bethesda, MD, ⁸Laboratory of Asthma and Lung Inflammation, Division of Intramural Research, NHLBI, NIH,, Bethesda, MD, ⁹Critical Care Medicine Department, Clinical Center, NIH, Bethesda, MD, ¹⁰University Hospitals of Geneva, Geneva, Switzerland, ¹¹NHLBI/NIH, Bethesda, MD, ¹²University of PIttsburgh, Pittsburgh, PA, ¹³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: Recently, pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS) have been reported in rare patients (pts) with systemic juvenile idiopathic arthritis (SJIA)…
Abstract Number: 1711 • ACR Convergence 2020
Musculoskeletal Sarcoidosis Learning Module for Internal Medicine Trainees: Developing a Rheumatology Curriculum
Kristen Fadel¹, Evan Dombrosky², Huzaefah Syed³, Joshua Gavin¹, Abhishek Nandan⁴, Stamatina Danielides⁵, Beth Rubinstein⁶, Yashswee KC⁷ and Sandra Johnson⁸, ¹Virginia Commonwealth University School of Medicine, Richmond, ²Virginia Commonwealth University School of Medicine, Glen Allen, VA, ³VCU Health, Richmond, VA, ⁴VCUHS and VA Medical Center- Richmond, VA, Richmond, VA, ⁵Virginia Commonwealth University School of Medicine, Richmond, VA, ⁶Virginia Commonwealth University, Richmond, VA, ⁷Virginia Commonwealth University School of Medicine, Henrico, VA, ⁸Virginia Commonwealth University Health Systems, Richmond
Background/Purpose: Sarcoidosis is a systemic, multi-organ disease frequently overlooked in the development of a differential diagnosis. Although typically considered a pulmonary disease, management decisions often fall to the Rheumatologist especially in the context of sarcoid-related arthritis or the initiation of immunosuppressive therapy. We…
Abstract Number: 0171 • ACR Convergence 2020
Interferon Response Gene Expression Differs in Whole Blood, Peripheral Blood Mononuclear Cells, Monocytes, T Cells, B Cells, and NK Cells in Patients with the Autoinflammatory Interferonopathies, CANDLE and SAVI
Jacob Mitchell¹, Sara Alehashemi², Bernadette Marrero³, Yan Huang⁴, Sofia Torreggiani¹, Lena Bichell¹, Gina Montealegre Sanchez⁵, Raphaela Goldbach-Mansky⁶ and Adriana de Jesus⁷, ¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Clarksville, MD, ³Computational Systems Biology Section/NIAID/NIH, Bethesda, MD, ⁴NIH, Bethesda, ⁵NIAID/NIH, Rockville, MD, ⁶Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD, ⁷Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD
Background/Purpose: The disease progression of patients (pts.) with type-I interferon (IFN)-mediated diseases undergoing treatment with JAK1 and JAK2 inhibitors is monitored in part by measuring…

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