Abstract Number: 1701 • ACR Convergence 2023
Treatment Regimens and Mortality in Systemic Sclerosis-associated Pulmonary Arterial Hypertension in Light of the 2022 ESC/ERS Guidelines
Background/Purpose: The 2022 ESC/ERS Guidelines recommend upfront combination therapy for low- and intermediate-risk, and triple therapy for high-risk patients with systemic sclerosis (SSc)-associated pulmonary arterial…Abstract Number: 2353 • ACR Convergence 2023
Role of Insulin-like Growth Factor Binding Protein-7 (IGFBP7) in Pulmonary Hypertension Pathogenesis and as a Biomarker Systemic Sclerosis-Associated Pulmonary Arterial Hypertension (SSc-PAH)
Background/Purpose: Systemic sclerosis (SSc, scleroderma) is an autoimmune connective tissue disease that causes autoimmunity, vascular dysfunction, and fibrosis of the skin, lungs, and other organs.…Abstract Number: 059 • 2023 Pediatric Rheumatology Symposium
Update of Clinical and Laboratory Features of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease (SJIA-LD) Cohort
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) associated lung disease (SJIA-LD) is an emerging and life-threatening clinical problem. Despite recent advances, there remain key unanswered questions…Abstract Number: 098 • 2023 Pediatric Rheumatology Symposium
Characterization of Pulmonary Nodules in Juvenile-onset Systemic Sclerosis: A Single Center Case-Series
Background/Purpose: Given the high prevalence of intestinal lung disease (ILD) in systemic sclerosis (SSc) and the low sensitivity of pulmonary function testing for diagnosing ILD,…Abstract Number: L16 • ACR Convergence 2022
Innovative Anti-pneumococcal Vaccine Strategies versus Standard Vaccination Regimen in Patients with ANCA-associated Vasculitides Receiving Rituximab Therapy: A Multicenter Randomized Controlled Trial (PNEUMOVAS)
Background/Purpose: Patients receiving glucocorticoids plus rituximab (RTX) show an increased risk of infection, especially invasive pneumococcal infections. Vaccine responses to influenza, Streptococcus pneumoniae and SARS-CoV-2…Abstract Number: 1049 • ACR Convergence 2022
Risk Stratification of Patients with Systemic Sclerosis-associated Pulmonary Arterial Hypertension in EUSTAR Using the Current and New Proposed Criteria
Background/Purpose: Pulmonary arterial hypertension (PAH) is a major clinical challenge in systemic sclerosis (SSc). A new definition for precapillary PH is proposed. Risk stratification is…Abstract Number: 1941 • ACR Convergence 2022
Pediatric Idiopathic Pulmonary Hemosiderosis – a Single-Center, Retrospective Study Comparing Use of Commonly Used Steroid-Sparing Agents
Background/Purpose: Idiopathic Pulmonary Hemosiderosis (IPH) is a rare, pediatric predominant condition causing episodic diffuse alveolar hemorrhage, which can be recurrent and life-threatening. Chronic steroid use…Abstract Number: 1057 • ACR Convergence 2022
Usefulness of REVEAL 2.0 Score as a Prognosis Tool of All-Cause Pulmonary Hypertension in Systemic Sclerosis Patients: A Multicentric Study
Background/Purpose: Pulmonary arterial hypertension (PAH) leads to a progressive right heart failure and death.PAH is a leading cause of death in Systemic Sclerosis (SSc). To…Abstract Number: 2160 • ACR Convergence 2022
The Role of CCL21 in Serum Samples from Systemic Sclerosis Patients
Background/Purpose: Chemokines appear to be important for the pathogenesis of autoimmune diseases. Humans may generate antibodies targeting chemokines leading to inhibition of signaling and driving…Abstract Number: 1058 • ACR Convergence 2022
Clinical Characteristics and Survival of Combined Pulmonary Arterial Hypertension and Interstitial Lung Disease in Systemic Sclerosis
Background/Purpose: Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) are the leading causes of systemic sclerosis (SSc) associated death. There is increasing interest in…Abstract Number: 2172 • ACR Convergence 2022
Pulmonary Function in Patients Diagnosed of Early Systemic Sclerosis: 10 Years-Experience
Background/Purpose: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc), often progressive and has a poor prognosis. A restrictive ventilatory defect could…Abstract Number: 1090 • ACR Convergence 2022
Pulmonary Function Testing (PFT) in Patients with Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis (AAV): Correlation with Radiologic Imaging
Background/Purpose: Although pulmonary manifestations occur frequently in AAV, empirical evidence of their impact on pulmonary function is scarce. This project analyzed PFT data from a…Abstract Number: 2175 • ACR Convergence 2022
Evaluation of the Distribution of Haemodynamic Parameters and Prognostic Impact in a Cohort of Patients with Systemic Sclerosis
Background/Purpose: Scleroderma (SSc) is a complex multisystem autoimmune disease, characterised by vasculopathy and fibrosis of skin and organs. Involvement of the cardiovascular system occurs frequently…Abstract Number: 1108 • ACR Convergence 2022
Abnormal Baseline Chest CT Shows Increased Risk for Immune Checkpoint Inhibitor Associated Pneumonitis
Background/Purpose: Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy but can result in off-target toxicities known as immune-related adverse events (irAEs). With increased use of…Abstract Number: 2176 • ACR Convergence 2022
Novel Tissue Turnover Blood Markers Are Associated with Pulmonary Arterial Hypertension and NYHA Stages in Patients with Systemic Sclerosis
Background/Purpose: Systemic sclerosis (SSc) is characterized by vasculopathy and fibrosis of the skin and internal organs. Cardiac involvement, pulmonary arterial hypertension (PAH), and interstitial lung…
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