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Introduction: Connective tissues diseases (CTDs) are a heterogeneous group of disorders that share certain clinical characteristics and disturbed immunoregulation. Interstitial lung diseases (ILDs), also known as diffuse parenchymal lung diseases, are among the most serious pulmonary complications associated with CTDs. Interleukin 9 (IL-9), IL-4 and interferon γ (IFN-γ) - cytokines with important roles in autoimmune disease - were studied in CTD patients and CTD-ILD patients.
Material And Methods: Sixty-one hospitalized untreated CTD patients were recruited, and 20 healthy volunteers were enrolled as controls. The 61 CTD patients were divided into a simple CTD group and a CTD-ILD group, and the plasma protein IL-9, IL-4 and IFN-γ levels were measured by enzyme-linked immunosorbent assay (ELISA).
Results: The results indicate that the serum IL-9 levels were significantly higher in CTD-ILD and simple CTD patients than they were in healthy controls (each p < 0.05) and that the levels were elevated in CTD-ILD patients compared with simple CTD patients (p < 0.05). The IL-4 levels were higher in CTD-ILD patients than they were in the simple CTD patients (p < 0.05) and healthy controls (p < 0.01). In addition, the serum IL-9 levels were negatively correlated with the level of IFN-γ (r (2) = 0.34, p = 0.01), the estimated percentage of predicted forced vital capacity (FVC%) (r (2) = 0.36, p = 0.00) and the estimated percentage of predicted diffusing capacity (DLCO%) (r (2) = 0.27, p = 0.04) and were positively correlated with the IL-4 level (r (2) = 0.31, p = 0.01).
Conclusions: Interleukin-9 may play an important role in the pathogenesis of CTD and may contribute to the progression of interstitial lung injury in CTD patients.
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http://dx.doi.org/10.5114/aoms.2015.47877 | DOI Listing |
JACC Case Rep
September 2025
Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois, USA; Northwestern Feinberg School of Medicine, Chicago, Illinois, USA.
Background: Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder (CTD) with musculoskeletal, craniofacial, and cardiovascular features with a prevalence of approximately 1:50,000. Morbidity and mortality often occur earlier in patients with LDS compared to patients with other CTDs.
Case Summary: We present a teenager with subacute heart failure, 4/6 holosystolic murmur with diastolic rumble, facial differences, and arachnodactyly.
J Cardiovasc Magn Reson
September 2025
Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
Introduction: Mitral annular disjunction (MAD) is a pathologic fibrous separation of the mitral valve hinge point from the ventricular myocardium. The aims of this study were to describe the range of MAD distance by cardiac magnetic resonance (CMR) in children and young adults with connective tissue disorders (CTDs) versus a healthy control sample, and to assess the MAD distance as a predictor of adverse cardiovascular outcomes.
Methods: This was a retrospective, single-center study of healthy subjects and patients with Marfan syndrome, Loeys-Dietz syndrome, Ehlers-Danlos syndrome, or nonspecific CTD who underwent CMR between 01/01/2000 and 01/01/2020.
Cureus
August 2025
Physiology, Alkindy College of Medicine, Baghdad University, Baghdad, IRQ.
Cor triatriatum is a rare congenital heart defect that divides the right or left atrium into three chambers. Although the diagnosis is typically made within the first years of birth, it can occasionally be made later in adulthood and is frequently associated with other cardiac defects but may be present in isolation. Clinical manifestations range from lung congestion, exhaustion, coughing, and dyspnea to the onset of heart failure.
View Article and Find Full Text PDFZhong Nan Da Xue Xue Bao Yi Xue Ban
May 2025
Department of Rheumatology and Immunology, Xiangya Hospital, Central South University, Changsha 410008.
Objectives: Patients with connective tissue diseases (CTD) have a high incidence of cardiac involvement, which often presents insidiously and can progress rapidly, making it one of the leading causes of death. Multiparametric cardiovascular magnetic resonance (CMR) provides a comprehensive quantitative evaluation of myocardial injury and is emerging as a valuable tool for detecting cardiac involvement in CTD. This study aims to investigate the correlations between CMR features and serological biomarkers in CTD patients, assess their potential clinical value, and further explore the impact of pre-CMR immunotherapy intensity on CMR-specific parameters, thereby evaluating the role of CMR in the early diagnosis of CTD-related cardiac involvement.
View Article and Find Full Text PDFEcotoxicol Environ Saf
September 2025
Jiangsu Key Laboratory of New Drug Research and Clinical Pharmacy, Xuzhou Medical University, Xuzhou, Jiangsu, China. Electronic address:
Pulmonary arterial hypertension (PAH) is a rare but serious disease that poses a significant threat to patients' quality of life and life expectancy. Perfluorooctane sulfonate (PFOS) is a widely used chemical that, due to its toxicity, persistence, and bioaccumulation properties, may increase the risk of developing PAH with prolonged exposure. This study investigated the molecular mechanisms linking PFOS exposure to PAH by integrating network toxicology and molecular docking methods.
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