Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 1345 • ACR Convergence 2022
Is Perceived Stress Associated with COVID-19 or Having a Systemic Rheumatic Disease?
Jonah Levine, Medha Barbhaiya, Vivian Bykerk, Deanna Jannat-Khah, DrPH, MSPH and Lisa Mandl, Hospital for Special Surgery, New York, NY
Background/Purpose: The SARS-CoV-2 pandemic is a worldwide mental health crisis. We evaluated perceived stress in patients seeking care for musculoskeletal conditions, and explored associations with…
Abstract Number: 1940 • ACR Convergence 2022
Genetic Variants and Cytokine Signatures in Patients with Syndrome of Undifferentiated Recurrent Fevers
Marci Macaraeg, Michael Matt, Grant Schulert, Elizabeth Baker and Elizabeth Handorf, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Unexplained, recurrent fevers are a common presentation to pediatric rheumatology and a cause of significant burden to affected families due to days of daycare…
Abstract Number: 0038 • ACR Convergence 2022
STING Antagonist (IFM4490) Inhibits STING Activation and STING Dependent Signal Transduction in Patients with STING-associated Vasculopathy with Onset in Infancy (SAVI)
Sachin Gaurav¹, Bin Lin², Sophia Park³, Shankar Venkatraman⁴, David Winkler⁴ and Raphaela Goldbach-Mansky⁵, ¹Translational Autoinflammatory Diseases Section, LCIM, NIAID,, Bethesda, MD, ²NIH, Bethesda, MD, ³NIAID, Bethesda, MD, ⁴IFM Therapeutics, Boston, MA, ⁵NIH/NIAID, Potomac, MD
Background/Purpose: SAVI is a rare, autoinflammatory type I interferonopathy caused by gain-of-function mutations that cause STING activation and excessive production of type I interferons and…
Abstract Number: 0785 • ACR Convergence 2022
Humoral Immune Responses Following Four SARS-CoV-2 Vaccine Doses or COVID-19 Infection in Patients with Immune-Mediated Inflammatory Diseases on Immunosuppressive Therapy: A Prospective Cohort Study
Hilde Ørbo¹, Kristin Hammersbøen Bjørlykke², Joe Sexton¹, Anne Therese Tveter¹, Ingrid Jyssum¹, Ingrid Egeland Christensen¹, David Warren³, Tore K. Kvien¹, Adity Chopra⁴, Grete Birkeland Kro³, Jørgen Jahnsen², Ludvig A. Munthe³, Espen Haavardsholm¹, Gunnveig Grødeland³, Sella Aarrestad Provan¹, Kristin Kaasen Jørgensen², Silje Watterdal Syversen¹ and Guro Løvik Goll¹, ¹Diakonhjemmet Hospital, Oslo, Norway, ²Akershus University Hospital, Oslo, Norway, ³Oslo University Hospital, Oslo, Norway, ⁴Oslo University Hospital, Oslo, Oslo, Norway
Background/Purpose: Patients with immune-mediated inflammatory diseases (IMIDs) on immunosuppressive therapy have impaired humoral immune responses to SARS-CoV-2 vaccination. This study aimed to assess serologic response…
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…

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