Abstract Number: 0459 • ACR Convergence 2020
Autologous Hematopoietic Stem Cell Transplantation for Behçet’s Disease: A Retrospective Survey of Patients Treated in Europe, on the Behalf of the Autoimmune Diseases Working Party of the European Society for Blood and Bone Marrow Transplantation
Background/Purpose: Behçet’s Disease (BD) is a rare autoimmune disease mostly presenting with recurrent oral and genital aphtosis, ulcers, and uveitis. Rare patients are refractory to…Abstract Number: 0460 • ACR Convergence 2020
Lymphocyte Clonal Expansion Distinguishes Different Forms of Uveitis
Background/Purpose: Anterior uveitis is a form of ocular inflammation associated with rheumatologic disease and can be categorized as granulomatous or non-granulomatous. Whether different ocular manifestations…Abstract Number: 0461 • ACR Convergence 2020
High-Throughput Single-Cell Analysis Reveals Unique Lung Cellular Subsets in a Murine Model of Rheumatoid Arthritis-Inflammatory Lung Disease
Background/Purpose: Rheumatoid arthritis (RA)-associated inflammatory lung disease is an extra-articular manifestation of RA associated with increased morbidity and mortality, whose precise molecular mechanisms remain undetermined.…Abstract Number: 0462 • ACR Convergence 2020
Lupus-like Autoimmunity and Increased Interferon Response in Patients with STAT3-deficient Hyper-IgE Syndrome
Background/Purpose: Autosomal dominant hyper-IgE syndrome (AD-HIES), also known as Job’s syndrome, is a rare primary immunodeficiency caused by dominant-negative loss-of-function (LOF) mutations in signal transducer…Abstract Number: 0463 • ACR Convergence 2020
Molecular Diagnosis of Childhood Immunodysregulation, Endocrinopathy and Enteropathy X-linked (IPEX)-Like Syndrome and Implications for Clinical Management
Background/Purpose: Patients with early-onset immunodysregulation, endocrinopathy and enteropathy but without identified mutations in FOXP3 are termed “IPEX-like,” and undergo trial-and-error immunosuppressive treatment with highly variable…Abstract Number: 0464 • ACR Convergence 2020
Assessment of the COVID-19 Pandemic from the Perspective of People with Rheumatic Musculoskeletal Diseases in Europe. Preliminary Results from the REUMAVID Study
Background/Purpose: The COVID-19 pandemic is an unprecedented public health crisis affecting people worldwide, including those with rheumatic and musculoskeletal diseases (RMDS). REUMAVID aims to assess…Abstract Number: 0465 • ACR Convergence 2020
The COVID-19 Pandemic and Its Effect on Patients with Chronic Rheumatologic Disease Regarding the Value of Vaccination Recommendations
Background/Purpose: Vaccinations are an important component of care in rheumatology as many patients are immunocompromised by treatment therapies. Patients are sometimes reticent to receive recommended…Abstract Number: 0466 • ACR Convergence 2020
Dosage Modification of Immunomodulatory Medications by Rheumatology Patients in New York City During the Peak of the COVID-19 Pandemic
Background/Purpose: Due to concerns about underlying immune dysregulation and immunosuppression, patients with systemic rheumatic diseases living in COVID-19 “hot spots” may have modified their immunomodulatory…Abstract Number: 0467 • ACR Convergence 2020
Incidence of COVID-19 in Patients Treated with Infliximab Compared to Patients Treated with Rituximab
Background/Purpose: The prevalence of anti-SARS-CoV-2 IgG infection was estimated at 9.7% in the Geneva population end of April 2020. (1) Immunosuppressed patients may be at…Abstract Number: 0468 • ACR Convergence 2020
Concerns and Health-Related Behaviors During the COVID-19 Pandemic in Patients with or Without Autoimmune Rheumatic Disease in a Large Physician Network
Background/Purpose: Patients with autoimmune rheumatic diseases (ARD) may be particularly concerned about COVID-19. We aimed to compare concerns and health-related behaviors of patients with common…Abstract Number: 0469 • ACR Convergence 2020
IFNγ Is Essential for Alveolar Macrophage Driven Lung Inflammation in Macrophage Activation Syndrome
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening cytokine storm syndrome frequently complicating systemic juvenile idiopathic arthritis (SJIA) and driven by IFNγ. MAS is also…Abstract Number: 0470 • ACR Convergence 2020
Th1 Polarization Defines the T Cell Compartment in the Joints of Oligoarticular Juvenile Idiopathic Arthritis Patients
Background/Purpose: Oligoarticular juvenile idiopathic arthritis (oligo JIA) is defined by limited joint involvement at disease onset. Some children achieve long-term remission while others continue to…Abstract Number: 0471 • ACR Convergence 2020
Splice Site Variants in IKBKG, Encoding NEMO, Detected by a Customized Analysis of Next-Generation Sequencing Data Cause an Early-onset Autoinflammatory Syndrome of Panniculitis and Cytopenias in Male and Female Patients
Background/Purpose: The Inhibitor of Kappa-B Kinase Regulatory Subunit Gamma (IKBKG) is located on the X chromosome and encodes the NF-κB essential modulator (NEMO). Loss-of-function mutations…Abstract Number: 0472 • ACR Convergence 2020
ApoB:ApoA1 Ratio Could Predict Atherosclerotic Risk in Juvenile-SLE Patients Associated with Altered Interferon Signalling in CD8+ T-cells
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease characterised by immune-dysregulation, chronic inflammation, type-I interferon (IFN) signatures and increased cardiovascular disease (CVD) risk…Abstract Number: 0473 • ACR Convergence 2020
Characterization of DOCK8 as a Novel Gene Associated with Cytokine Storm Syndrome
Background/Purpose: Cytokine storm syndrome (CSS), also known as macrophage activation syndrome (MAS) or secondary hemophagocytic lymphohistiocytosis (HLH), is a life threatening condition that commonly presents…
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