Systemic sclerosis is an autoimmune connective tissue disorder, which can be progressive with multisystem involvement. Guidance on the management of complications is based on a limited data set and practice amongst clinicians can vary. The UK Scleroderma study group set up several working groups to agree some consensus pathways for the management of specific complications. Approximately nine out of ten patients with systemic sclerosis will have involvement of the gastrointestinal system and in this review article we explore the management of these complications in a symptom-based approach. The algorithms are a useful tool for clinicians, which we hope, will be a point of reference and highlight the need for further research in these areas.
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Rev Mal Respir
January 2025
Service de médecine interne, hôpital Habib Thameur, faculté de médecine de Tunis, université de Tunis El Manar, Tunis, Tunisie.
Otherwise known as mediastinal emphysema, pneumomediastinum (PNM) in connective tissue diseases is a rare clinical entity. Few cases have been described in the literature. In fact, it only exceptionally complicates the evolution of connective tissue diseases.
View Article and Find Full Text PDFCurr Cardiol Rep
January 2025
Johns Hopkins University Division of Cardiology, Baltimore, MD, USA.
Purpose Of Review: The present review aims to address systemic sclerosis (SSc)-associated myocardial disease, a significant cause of morbidity and mortality, by examining the mechanisms of inflammation, microvascular dysfunction, and fibrosis that drive cardiac involvement. The objective is to elucidate critical risk factors and explore advanced diagnostic tools for early detection, enhancing patient outcomes by identifying those at highest risk.
Recent Findings: Recent studies underscore the importance of specific autoantibody profiles, disease duration, and cardiovascular comorbidities as key risk factors for severe cardiac manifestations in SSc.
Rheumatol Int
January 2025
Division of Hematology-Oncology, Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, AR, 72205, USA.
Chimeric antigen receptor T-cell (CAR-T) therapy has revolutionized the treatment of various hematological malignancies. Recently, CAR-T has been used in refractory auto-immune diseases with initial encouraging results. In this systematic review, we examined the safety and efficacy of CAR-T in patients with refractory auto-immune diseases.
View Article and Find Full Text PDFSci Rep
January 2025
Harbin Medical University, Harbin, Heilongjiang Province, China.
Interstitial lung disease (ILD) is known to be a major complication of systemic sclerosis (SSc) and a leading cause of death in SSc patients. As the most common type of ILD, the pathogenesis of idiopathic pulmonary fibrosis (IPF) has not been fully elucidated. In this study, weighted correlation network analysis (WGCNA), protein‒protein interaction, Kaplan-Meier curve, univariate Cox analysis and machine learning methods were used on datasets from the Gene Expression Omnibus database.
View Article and Find Full Text PDFNat Genet
January 2025
Department of Statistical Genetics, Osaka University Graduate School of Medicine, Suita, Japan.
Aberrant immune responses to viral pathogens contribute to pathogenesis, but our understanding of pathological immune responses caused by viruses within the human virome, especially at a population scale, remains limited. We analyzed whole-genome sequencing datasets of 6,321 Japanese individuals, including patients with autoimmune diseases (psoriasis vulgaris, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), pulmonary alveolar proteinosis (PAP) or multiple sclerosis) and coronavirus disease 2019 (COVID-19), or healthy controls. We systematically quantified two constituents of the blood DNA virome, endogenous HHV-6 (eHHV-6) and anellovirus.
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