Progressive systemic sclerosis (PSS), a disease still of unknown origin, is a generalized autoimmune disorder characterized by immunological abnormalities, microvascular dysfunction, and tissue fibrosis. The mechanism leading to selective microvascular injury in PSS is not completely known, however it is now clear that neuropeptides, vascular endothelium, and disturbances in the regulation of fibroblast function are the three major contributors to the increased fibrosis of skin and internal organs. Thus, endothelial cell and fibroblast dysfunction may be linked through the paracrine activity of soluble endothelial cell products: the cytokine cascade (IL-1, TGF-beta-1, PDGF, TNF, etc.). In systemic sclerosis, the exaggerated generalized vasospastic tendency is clinically represented by Raynaud's phenomenon as shown by an early digital arterial closure after cold stimulation, and by an inadequate vasodilatory response to heat. In this review we summarize recently established data that center around the role of adhesion molecules, immune reactions, and aberrant fibroblast biology and metabolism in effecting vascular and connective tissue alterations in this disease. Only a better knowledge of the pathophysiological process involved in scleroderma might lead to the development of new therapeutic approaches.
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Eur Heart J Cardiovasc Imaging
January 2025
Department of Cardiovascular Medicine, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.
Aims: Left ventricular (LV) diastolic dysfunction and heart failure with preserved ejection fraction (HFpEF) are common cardiac complications of patients with systemic sclerosis (SSc). Exercise stress echocardiography is often used in symptomatic patients with SSc to detect abnormal increases in pulmonary pressures during exercise, but the pathophysiologic and prognostic significance of exercise stress echocardiography to assess the presence of HFpEF in these patients is unclear.
Methods And Results: Patients with SSc (n=140) underwent ergometry exercise stress echocardiography with simultaneous expired gas analysis.
Cureus
December 2024
Department of Oral Medicine, Nihon University School of Dentistry, Tokyo, JPN.
Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome, a systemic sclerosis subtype, features skin thickening, vascular issues, and organ involvement, causing complications in the gastrointestinal and musculoskeletal systems. Herein, we present a rare case of oral candidiasis, with CREST syndrome. The patient presented with xerostomia, tongue erythema, and burning pain.
View Article and Find Full Text PDFACS Nano
January 2025
Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing 210008, China.
Cyclophosphamide has a certain therapeutic effect on treating systemic sclerosis (SSc), while difficulties exist in controlling severe systematic side effects and enhancing targeting capacity. Here, inspired from the natural extracellular matrix composition, we propose a cyclophosphamide-encapsulated nanogel based on dendritic polymers polyamidoamine (PAMAM) for SSc treatment. We combine bovine serum albumin and generation 5 (G5) PAMAM dendrimers with polyphenol modification to obtain nanogels featured with antioxidant and anti-inflammatory effects.
View Article and Find Full Text PDFLupus Sci Med
January 2025
Amsterdam UMC Locatie VUmc, Amsterdam, The Netherlands.
CD19-directed chimeric antigen receptor (CAR) T-cell therapy, originally developed for haematological malignancies, has recently emerged as a promising therapy for patients with autoimmune diseases. By selectively depleting CD19-positive B-cells, this therapy brings a new approach in resetting immune dysregulation and potentially providing long-term remission for patients with a refractory disease. Recent reports have highlighted its effectiveness in conditions such as SLE, systemic sclerosis and myositis.
View Article and Find Full Text PDFClin Rheumatol
January 2025
Rheumatology Unit, Scleroderma Unit, University Hospital of Modena, Via del Pozzo, 71-41125, Modena, Italy.
The aims of this study were to investigate the prevalence of cryofibrinogenemia in a cohort of patients with systemic sclerosis (SSc) regardless of clinical manifestations, who were admitted to our hospital and determine the associations among CF positivity, disease features and ongoing therapies. This was a monocentric and retrospective study. The inclusion criteria were a diagnosis of SSc (according to the ACR/EULAR 2013 classification criteria), regular administration of i.
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