Effects of nintedanib in patients with systemic sclerosis-associated ILD (SSc-ILD) and differing extents of lung fibrosis: the SENSCIS trial*

2020 ◽  
Author(s):  
R Wiewrodt ◽  
G Raghu ◽  
O Distler ◽  
A Azuma ◽  
KB Highland ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lung Fibrosis

The involvement of leucine-rich α-2 glycoprotein in the progression of skin and lung fibrosis in bleomycin-induced systemic sclerosis model

2021 ◽  
pp. 1-10
Author(s):  
Hideki Nakajima ◽  
Kimiko Nakajima ◽  
Satoshi Serada ◽  
Minoru Fujimoto ◽  
Tetsuji Naka ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lung Fibrosis

Differences in Texture Analysis Parameters Between Active Alveolitis and Lung Fibrosis in Chest CT of Patients with Systemic Sclerosis

2017 ◽  
Vol 24 (12) ◽  
pp. 1596-1603 ◽  
Author(s):  
Christopher Kloth ◽  
Anya C. Blum ◽  
Wolfgang M. Thaiss ◽  
Heike Preibsch ◽  
Hendrik Ditt ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Texture Analysis ◽  
Lung Fibrosis ◽  
Chest Ct

scholarly journals The JAK/STAT pathway is activated in systemic sclerosis and is effectively targeted by tofacitinib

2019 ◽  
Vol 5 (1) ◽  
pp. 40-50 ◽  
Author(s):  
Wenxia Wang ◽  
Swati Bhattacharyya ◽  
Roberta Goncalves Marangoni ◽  
Mary Carns ◽  
Kathleen Dennis-Aren ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lung Fibrosis ◽  
Bioinformatic Analysis ◽  
Jak Inhibitor ◽  
Stat3 Phosphorylation ◽  
Fibrotic Diseases ◽  
Lung Biopsies ◽  
Organ Fibrosis ◽  
Substantial Morbidity ◽  
Stat Pathway

Rationale: Fibrosis leads to failure of the skin, lungs, and other organs in systemic sclerosis; accounts for substantial morbidity and mortality; and lacks effective therapy. Myofibroblast activation underlies organ fibrosis, but the key extracellular cues driving persistence of the process remain incompletely characterized. Objectives: The objectives were to evaluate activation of the IL6/JAK/STAT axis associated with fibrosis in skin and lung biopsies from systemic sclerosis patients and effects of the Food and Drug Administration–approved JAK/STAT inhibitor, tofacitinib, on skin and lung fibrosis in animal models. Methods: Bioinformatic analysis showed that IL6/JAK/STAT3 and tofacitinib gene signatures were aberrant in biopsies from systemic sclerosis patients in four independent cohorts. The results were confirmed by JAK and STAT3 phosphorylation in both skin and lung biopsies from patients with systemic sclerosis. Furthermore, treatment of mice with the selective JAK inhibitor tofacitinib not only prevented bleomycin-induced skin and lung fibrosis but also reduced skin fibrosis in TSK1/+ mice. Conclusion: These findings implicate the JAK/STAT pathway in systemic sclerosis skin and lung fibrosis and identify tofacitinib as a potential antifibrotic agent for the treatment of systemic sclerosis and other fibrotic diseases.

Effects of nintedanib in patients with systemic sclerosis-associated ILD (SSc-ILD) and differing extents of lung fibrosis: the SENSCIS trial

2019 ◽  
Author(s):  
Ganesh Raghu ◽  
Oliver Distler ◽  
Arata Azuma ◽  
Aryeh Fischer ◽  
Kristin B. Highland ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lung Fibrosis

scholarly journals Circulating Tissue Inhibitor of Matrix Metalloproteinase-4 (TIMP-4) in Systemic Sclerosis Patients with Elevated Pulmonary Arterial Pressure

2008 ◽  
Vol 2008 ◽  
pp. 1-8 ◽  
Author(s):  
Gialafos J. Elias ◽  
Moyssakis Ioannis ◽  
Psaltopoulou Theodora ◽  
Papadopoulos P. Dimitrios ◽  
Perea Despoina ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lung Fibrosis ◽  
Pulmonary Function Tests ◽  
Systolic Pressure ◽  
Serum Levels ◽  
Cross Sectional Study ◽  
Pulmonary Artery Systolic Pressure ◽  
Cross Sectional ◽  
Skin Sclerosis ◽  
Pulmonary Arterial

Decreased levels of matrix metalloproteinases (MMPs) or excess levels of their tissue inhibitors (TIMPs) may contribute to dysregulation of extracellular matrix turnover in systemic sclerosis (SSc). In a cross-sectional study of 106 SSc patients, we measured serum levels of TIMP-4 which is preferentially expressed in cardiovascular structures and searched for correlations with simultaneously performed echocardiography measurements of pulmonary artery systolic pressure (PASP), myocardial performance, and pulmonary function tests. TIMP-4, but not MMP-9, levels were significantly raised in patients with SSc than controls. However, in the subgroup of patients with PASP measurements lower to 40 mmHg (n=69), TIMP-4 levels were comparable to controls irrespective of the presence of diffuse or limited skin involvement, or lung fibrosis. Individual PASP measurements suggestive of pulmonary hypertension were associated with increased TIMP-4 serum levels (P=.03), independently of age, extent of skin sclerosis, or lung fibrosis, suggesting a cardiopulmonary vasculature-specific role of TIMP-4 activation in SSc.

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