Systemic sclerosis (SSc) is a generalized connective tissue disorder, and SSc patients are at risk of developing pulmonary arterial hypertension (PAH). The aims of this study are to evaluate the right ventricular regional systolic function using two-dimensional speckle-tracking echocardiography (2D STE) and to determine the predictive ability of peak longitudinal systolic strain (PLSS) at the RV lateral wall for PAH in SSc patients. 80 SSc patients (mean age 51 ± 12 years) were included in the study. Echocardiography and 2D STE were performed at baseline and after 12 months. RHC was performed only in SSc patients with clinical indications. PLSS at the apical segment of the RV free wall was significantly impaired in PAH patients compared with non-PH patients (-14.6 ± 5.9 vs. - 22.2 ± 7.5%, p = 0.034). PLSS at the basal, mid, and apical segments of the RV free wall was lower in both groups at follow-up compared to baseline, but the drop in strain values was statistically significant only in the non-PH group (p < 0.05). Right atrial area (OR 1.758; p = 0.023), peak tricuspid regurgitation velocity (OR 24.23; p = 0.011) and PLSS at the apical segment of the RV lateral wall (OR 2.47; p = 0.005) were independent predictors of PAH. A cut-off value of - 14.48% PLSS at the apical segment of the RV lateral wall resulted in 100% specificity for predicting PAH in SSc patients. RV pressure overload affects RV systolic function as manifested by impaired RV longitudinal deformation. Evaluating RV regional systolic function with 2D STE could be useful as an additional echocardiographic parameter for screening PAH in SSc patients.
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http://dx.doi.org/10.1007/s10554-018-1299-z | DOI Listing |
Rev Cardiovasc Med
January 2025
Division of Cardiology, Maggiore della Carità Hospital, 28100 Novara, Italy.
Cardiac manifestations in systemic sclerosis (SSc) are variable and are associated with a poor prognosis, frequently resulting in impaired right ventricular function and heart failure. A high proportion of patients with SSc experience pulmonary arterial hypertension (PAH), interstitial lung disease, or myocardial involvement, all of which can lead to exercise intolerance. In this context, cardiopulmonary exercise testing (CPET) is a useful tool for diagnosing exercise intolerance, elucidating its pathophysiology, and assessing its prognosis.
View Article and Find Full Text PDFRMD Open
January 2025
Epidemiology and Health Services Research, German Rheumatology Research Center, Berlin, Germany.
Objective: To analyse work participation among patients with inflammatory rheumatic musculoskeletal diseases (iRMDs), namely rheumatoid arthritis (RA), axial spondyloarthritis (axSpA), psoriatic arthritis (PsA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and ANCA-associated vasculitis (AAV).
Methods: A cross-sectional sample of 16 421 patients from the National Database of the German Collaborative Arthritis Centers, aged <65 years were analysed. For each diagnosis, yearly rates of absenteeism, employment and disability pensions were analysed from 2010 to 2022.
J Clin Med
January 2025
Rheumatology Unit, Department of Precision and Regenerative Medicine Jonian Area (DiPReMeJ), University of Bari "Aldo Moro", 70124 Bari, Italy.
Pulmonary arterial hypertension (PAH) is a complication of systemic sclerosis (SSc), and several screening algorithms have been proposed for the early detection of PAH in SSc. This study aimed to evaluate the predicting values of the DETECT algorithm for SSc-PAH screening in patients with SSc undergoing right heart catheterization (RHC) based on 2015 ESC/ERS echocardiographic criteria in a real-life setting. Patients fulfilling the 2013 ACR/EULAR classification criteria for SSc and with available data for PAH screening with the DETECT algorithm and the 2015 ESC/ERS echocardiographic criteria were retrospectively enrolled from January to June 2017 and then followed for 5 years.
View Article and Find Full Text PDFInt 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 PDFMedicina (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.
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