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Exploring CAR T-Cell Dynamics: Balancing Potent Cytotoxicity and Controlled Inflammation in CAR T-Cells Derived from Systemic Sclerosis and Myositis Patients.
Int J Mol Sci
January 2025
Department of Internal Medicine 5, Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, 91054 Erlangen, Germany.
Systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and idiopathic inflammatory myositis (IIM) are autoimmune diseases managed with long-term immunosuppressive therapies. Hu19-CD828Z, a fully human anti-CD19 chimeric antigen receptor (CAR) with a CD28 costimulatory domain, is engineered to potently deplete B-cells. In this study, we manufactured Hu19-CD828Z CAR T-cells from peripheral blood of SLE, IIM, and SSc patients and healthy donors (HDs).
View Article and Find Full Text PDFMultimodal Screening for Pulmonary Arterial Hypertension in Systemic Scleroderma: Current Methods and Future Directions.
Medicina (Kaunas)
December 2024
Department of Rheumatology and Physiotherapy, "Grigore T. Popa" University of Medicine and Pharmacy, 16 University Street, 700115 Iasi, Romania.
Systemic sclerosis (SSc) is an immuno-inflammatory rheumatic disease that can affect both the skin and internal organs through fibrosis. Pulmonary arterial hypertension (PAH) is one of the most severe secondary complications. Structural changes in the vascular bed lead to increased pressures in the pulmonary circulation, severely impacting the right heart and significantly affecting mortality.
View Article and Find Full Text PDFAutomated AI-based image analysis for quantification and prediction of interstitial lung disease in systemic sclerosis patients.
Respir Res
January 2025
National Heart and Lung Institute, Imperial College London, London, UK.
Background: Systemic sclerosis (SSc) is a rare connective tissue disease associated with rapidly evolving interstitial lung disease (ILD), driving its mortality. Specific imaging-based biomarkers associated with the evolution of lung disease are needed to help predict and quantify ILD.
Methods: We evaluated the potential of an automated ILD quantification system (icolung) from chest CT scans, to help in quantification and prediction of ILD progression in SSc-ILD.
ORAL MANIFESTATIONS IN JUVENILE SCLERODERMA: CLINICAL PRESENTATIONS AND HISTOPATHOLOGICAL CHARACTERISTICS.
Georgian Med News
November 2024
Juvenile scleroderma (JS) is a rare chronic connective tissue disorder characterized by stiffening of the skin and soft tissues, including the oral cavity and perioral tissues, leading to fibrosis and a large spectrum of internal organs involvement, cosmetic defects, and early infant disability. The aim of this study was to investigate the histomorphological features of lesions of oral mucosa tissues in children with juvenile scleroderma (JS). 39 JS patients (9 with juvenile systemic sclerosis - JSS and 20 with juvenile scleroderma of head-JSH aged from 5 to 17 years were observed with dental examination and morphological investigation of the dental mucosa.
View Article and Find Full Text PDFAbnormal Esophageal Scintigraphy Associates With a Distinct Clinical Phenotype in Patients With Systemic Sclerosis.
ACR Open Rheumatol
January 2025
UTHealth Houston, Houston, Texas.
Objective: In systemic sclerosis (SSc), absent contractility (AC) rather than ineffective esophageal motility on manometry is associated with a severe esophageal and extraintestinal phenotype. We sought to determine whether slow esophageal transit on scintigraphy associates with a comparable clinical phenotype to that of AC on manometry, as scintigraphy may serve as a noninvasive approach to risk-stratify patients with SSc.
Methods: Clinical, demographic, and serologic features were compared between patients with and without delayed esophageal transit on scintigraphy.
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