Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 1568 • ACR Convergence 2022
Evaluation for Allogeneic Hematopoietic Stem Cell Transplant Indications and Eligibility in a Prospective Cohort of Patients with VEXAS
Marcela Ferrada¹, Peter Grayson², Lorena Wilson³, David Beck⁴, Wendy Goodspeed⁵, Ivana Darden⁶, Emma Groarke⁶, Dennis Hickstein⁶ and Bhavisha Patel⁷, ¹National Institutes of Health, Bethesda, MD, ²National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ³National Institutes of Health, Silver Spring, MD, ⁴New York University, New York, NY, ⁵National Institutes of Health (NIH), Bethesda, MD, ⁶NIH/NHLBI, Bethesda, MD, ⁷National Institutes of Health, Beltsville, MD
Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly described genetic disease due to mutations in UBA1 in hematopoietic stem cells. Patients…
Abstract Number: 1942 • ACR Convergence 2022
Maybe Silent: Spine Disease in Chronic Recurrent Multifocal Osteomyelitis, Clinical Features, Risk Factors, and Red Flags
Shima Yasin¹, T. Shawn Sato², Emma Leisinger², Aleksander Lenert², Yongdong (Dan) Zhao³ and Polly Ferguson¹, ¹University of Iowa Carver College of Medicine, Iowa City, IA, ²University of Iowa, Iowa City, IA, ³University of Washington, Redmond, WA
Background/Purpose: Chronic recurrent multifocal osteomyelitis (CRMO) is a female predominant autoinflammatory bone disease. The average age at disease onset is 9-10 years. The majority have…
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…
Abstract Number: 0130 • ACR Convergence 2022
Three Chinese Pedigrees of A20 Haploinsufficiency
Yi Tian¹, Min Shen², Bingxuan Wu², linyi Peng² and Jian Wang³, ¹Rheumatology Department, The Second Affiliated Hospital of Dalian Medical University, Dalian, China, ²Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China, ³Jinzhou City Center for Disease Control and Prevention, Jinzhou, China
Background/Purpose: Haploinsufficiency of A20 (HA20) is a newly described rare autoinflammatory disease caused by TNFAIP3 gene mutations. HA20 has been seldom documented in the Chinese…
Abstract Number: 0791 • ACR Convergence 2022
Immunogenicity and Safety of a Three-dose SARS-CoV-2 Vaccination Strategy in Patients with Immune-mediated Inflammatory Diseases on Immunosuppressive Therapy
Ingrid Jyssum¹, Anne Therese Tveter¹, Joe Sexton¹, Ingrid Egeland Christensen¹, Siri Mjaaland², David Warren³, Tore K. kvien¹, Kristin Hammersbøen Bjørlykke⁴, Grete Birkeland Kro³, Jørgen Jahnsen⁴, Ludvig A. Munthe³, Espen Haavardsholm¹, Gunnveig Grødeland³, Sella Aarrestad Provan¹, Kristin Kaasen Jørgensen⁴, Guro Goll¹ and Silje Watterdal Syversen¹, ¹Diakonhjemmet Hospital, OSLO, Norway, ²Norwegian Institute of Public Health, Oslo, Norway, ³Oslo University Hospital, Oslo, Norway, ⁴Akershus University Hospital, Oslo, Norway
Background/Purpose: Humoral vaccine responses to SARS-Cov-2 vaccines are impaired and short lasting in patients with immune-mediated inflammatory diseases (IMIDs). Concerns have been raised regarding their…
Abstract Number: 1698 • ACR Convergence 2022
LINE1 Reverse Transcriptase Inhibitors Abrogate Type 1 Interferon Responses
Daniel Rios¹, Nafeeza Hafeez¹, Brian Desrosiers¹, Shradha Rao¹, Jess Floro¹, Joelle Baddour¹, Nisha Perez², Greg Bisacchi², Oliver Saunders², Donna Romero¹, Menachem Fromer², Enrique Garcia-Rivera², Wilson McKerrow², Julie Behr², Bryan Thornlow², Dennis Zaller², David Proia² and Wenyan Miao², ¹Rome Therapeutics, Cambridge, MA, ²Rome Tx, Cambridge, MA
Background/Purpose: Transposable elements (TEs) are mobile DNA elements that can replicate and move from one position to another within the host genome. Through co-evolution, TEs…
Abstract Number: 2101 • ACR Convergence 2022
Outcomes of Autoimmune Hemolytic Anemia in Patients with Systemic Lupus Erythematosus: A Nationwide Inpatient Sample Study
Ekrem Turk, Vaishali Deenadayalan, Khaldun Obeidat and Pierre Rodriguez, John H. Stroger, Jr. Hospital of Cook County, Chicago, IL
Background/Purpose: Autoimmune Hemolytic Anemia (AIHA) is one of the manifestations of Systemic lupus erythematosus (SLE), an autoimmune connective tissue disease. AIHA may be present in…
Abstract Number: 0134 • ACR Convergence 2022
Combination Treatment with Baricitinib and Pulse Steroids in Severe COVID19: A
Francesco Ferro¹, Elena Elefante², Italiano Nazzareno², Gaetano La Rocca³, Davide Schilirò², Michele Moretti², Roberto Mozzo⁴, De Simone Luigi⁴, Chiara Baldini⁵ and Marta Mosca², ¹Clinical and Experimental Medicine Department, Azienda Ospedaliero-Universitaria Pisana, Pisa, Pisa, Italy, ²University of Pisa, Pisa, Italy, ³University of Pisa, Rheumatology Unit, Palermo, Palermo, Italy, ⁴Anesthesiology & Intensive Care Unit/Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy, ⁵University of Pisa, Pisa, Pisa, Italy
Background/Purpose: Growing evidence from RCTs and real-life experiences has highlighted important similarities between severe COVID19 and rapidly progressive interstitial lung diseases (RP-ILD) occurring in connective…
Abstract Number: 0812 • ACR Convergence 2022
3-years Safety and Efficacy Outcomes of Canakinumab Treatment in Cryopyrin-associated Periodic Syndromes (CAPS) – Data from the RELIANCE Registry
Jasmin B. Kuemmerle-Deschner¹, Birgit Kortus-Goetze², Prasad T. Oommen³, Ales Janda⁴, Juergen Rech⁵, Catharina Schuetz⁶, Tilmann Kallinich⁷, Frank Weller-Heinemann⁸, Gerd Horneff⁹, Ivan Foeldvari¹⁰, Florian Meier¹¹, Michael Borte¹², Tobias Krickau¹³, Julia Weber-Arden¹⁴ and Norbert Blank¹⁵, ¹Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, Germany, ²Division of Nephrology, University of Marburg, Marburg, Germany, ³Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁴Department of Pediatrics, University Hospital Ulm, Ulm, Germany, ⁵University Clinic Erlangen, Erlangen, Germany, ⁶Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, ⁷Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ⁸Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ⁹Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ¹⁰Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ¹¹Division 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, ¹²ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Germany, Leipzig, Sachsen, Germany, ¹³Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Pediatrics, Erlangen, Germany, ¹⁴Novartis Pharma GmbH, Nuernberg, Germany, ¹⁵Rheumatology, University Hospital Heidelberg, Heidelberg, Germany
Background/Purpose: Cryopyrin-associated periodic syndromes (CAPS) are monogenic autoinflammatory diseases with severe systemic inflammation. The IL-1β inhibitor canakinumab (CAN) leads to a rapid remission of CAPS…
Abstract Number: 1701 • ACR Convergence 2022
High TNFAIP3-Transfected Cell Activity: An In Vitro Model for Phagocyte Study of Monogenic Behçet’s Disease Pathophysiology
Patricia Pontes Aires¹, André Cunha¹, DANIELA GERENT PETRY PIOTTO¹, Maria Teresa Terreri² and Sandro Perazzio¹, ¹Universidade Federal de São Paulo, São Paulo, Brazil, ²Universidad Federal São Paulo, São Paulo, Brazil
Background/Purpose: A20 haploinsufficiency (A20HA), an important monogenic etiology of Behçet's disease (BD), is caused by loss-of-function mutations in TNFAIP3. Although monocytes play an important role…
Abstract Number: 2194 • ACR Convergence 2022
Is Vaccination Against Covid-19 Associated with Autoimmune Rheumatic Disease Flare? Self-controlled Case Series Analysis Using Data from the Clinical Practice Research Datalink (Aurum)
Georgina Nakafero¹, Matthew J. Grainge¹, Tim Card¹, Christian Mallen², Jonathan S. Nguyen van Tam¹, Hywel C. Williams¹ and Abhishek Abhishek¹, ¹University of Nottingham, Nottingham, United Kingdom, ²Keele University, Keele, United Kingdom
Background/Purpose: It is unclear whether autoimmune rheumatic disease (AIRD) flares are associated with the recent prior Covid-19 vaccination, and whether the association was influenced by…
Abstract Number: 0138 • ACR Convergence 2022
Patients with Familial Mediterranean Fever (FMF) Under Canakinumab Treatment – Long-term Efficacy and Safety Interim Data of the RELIANCE Registry
Joerg Henes¹, Jasmin B. Kuemmerle-Deschner², Tobias Krickau³, Tilmann Kallinich⁴, Frank Dressler⁵, Gerd Horneff⁶, Florian Meier⁷, Ivan Foeldvari⁸, Frank Weller-Heinemann⁹, Birgit Kortus-Goetze¹⁰, Markus Hufnagel¹¹, Juergen Rech¹², Prasad T. Oommen¹³, Julia Weber-Arden¹⁴ and Norbert Blank¹⁵, ¹Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, ²Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, Germany, ³Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Pediatrics, Erlangen, Germany, ⁴Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ⁵Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, ⁶Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ⁷Division 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, ⁸Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ⁹Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ¹⁰Division of Nephrology, University of Marburg, Marburg, Germany, ¹¹Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, ¹²University Clinic Erlangen, Erlangen, Germany, ¹³Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ¹⁴Novartis Pharma GmbH, Nuernberg, Germany, ¹⁵Rheumatology, University Hospital Heidelberg, Heidelberg, Germany
Background/Purpose: Familial Mediterranean fever (FMF) is a chronic disease characterized by recurrent episodes of fever and serositis, with a risk of severe complications (e. g.…

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