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…Abstract Number: 0075 • ACR Convergence 2022
Real-World Treatment Patterns Among Patients with Connective Tissue Disorder–Related Pulmonary Arterial Hypertension in the United States: A Retrospective Claims-Based Analysis
Background/Purpose: Connective tissue disorders (CTDs) are the most frequent diseases associated with pulmonary arterial hypertension (PAH), accounting for 11% – 28% of PAH cases. A…Abstract Number: 1110 • ACR Convergence 2022
Solving Sarcoidosis: A Transcriptome-based Meta-analysis of Clinical Sarcoidosis Studies Illustrates Shared Pathophysiology, Identifies Candidate Biomarkers and Suggests a Therapeutic Mechanism of JAK Inhibition
Background/Purpose: Sarcoidosis is a systemic, non-caseating granulomatous disease driven by a dysregulated immune response to environmental antigens. A wide range of clinical manifestations coupled with…Abstract Number: 0124 • ACR Convergence 2022
Evaluation of Comorbidity Burden and Its Effect on Lung Disease Progression and Mortality in a Cohort of Patients with Interstitial Pneumonia with Autoimmune Features
Background/Purpose: Interstitial pneumonia with autoimmune features (IPAF) is a subset of interstitial lung disease (ILD) which manifests with interstitial pneumonia and features of autoimmunity, yet…Abstract Number: 1282 • ACR Convergence 2022
Practice Patterns for Pneumocystis Jiroveci Pneumonia (PJP) Prophylaxis Among Nephrologists and Rheumatologists – an International Survey Study
Background/Purpose: Patients with autoimmune disorders such as systemic lupus erythematosus (SLE), lupus nephritis (LN) and granulomatosis with polyangiitis (GPA) frequently require treatment with immunosuppressive drugs.…Abstract Number: 0140 • ACR Convergence 2022
Continued Treatment with Nintedanib in Patients with Progressive Fibrosing Autoimmune Disease-Related Interstitial Lung Diseases (ILDs): Data from INBUILD-ON
Background/Purpose: In the INBUILD trial in patients with progressive fibrosing ILDs other than idiopathic pulmonary fibrosis, nintedanib reduced the rate of decline in forced vital…
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