Abstract Number: 1936 • ACR Convergence 2022
A De Novo Dominant-negative PSMB8 mutation is Linked to a More Severe CANDLE-like Phenotype
Background/Purpose: Homozygote (HM)/compound heterozygote (CH) and digenic (DG) mutations in proteasome subunits cause the autoinflammatory syndrome CANDLE, or proteasome-associated autoinflammatory syndrome (PRAAS). Inflammation is mediated…Abstract Number: 0021 • ACR Convergence 2022
DB-2306, a Novel Anti-TNFα Monoclonal Antibody Drug Conjugate, Is a Promising Novel Therapeutic Approach for Autoimmune Disease
Background/Purpose: Small molecule immune modulators, such as glucocorticoids (GCs), are highly effective in treatment for various inflammatory diseases. However, prolonged systemic administration of GCs is…Abstract Number: 0735 • ACR Convergence 2022
Facilitators and Barriers to Weekly Monitoring of Disease Activity with Electronic Patient Reported Outcomes: A Focus Group Study of Patients with Inflammatory Arthritis
Background/Purpose: Telemonitoring disease activity with electronic patient reported outcomes (ePRO's) may reduce the workload of rheumatic care by decreasing outpatient clinic visits. However, low adherence…Abstract Number: 1345 • ACR Convergence 2022
Is Perceived Stress Associated with COVID-19 or Having a Systemic Rheumatic Disease?
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
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)
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
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
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
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
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
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
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
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
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
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…
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