Antifibrotic activities of pirfenidone in animal models
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TLDR
The consistent antifibrotic activity of pirfenidone in a broad array of animal models provides a strong preclinical rationale for the clinical characterisation of Pirfenidones in pulmonary fibrosis and, potentially, other conditions with a significant fibrotic component.Abstract:
Pirfenidone is an orally active small molecule that has recently been evaluated in large clinical trials for the treatment of idiopathic pulmonary fibrosis, a fatal disease in which the uncontrolled deposition of extracellular matrix leads to progressive loss of lung function This review describes the activity of pirfenidone in several well-characterised animal models of fibrosis in the lung, liver, heart and kidney In these studies, treatment-related reductions in fibrosis are associated with modulation of cytokines and growth factors, with the most commonly reported effect being reduction of transforming growth factor-β The consistent antifibrotic activity of pirfenidone in a broad array of animal models provides a strong preclinical rationale for the clinical characterisation of pirfenidone in pulmonary fibrosis and, potentially, other conditions with a significant fibrotic componentread more
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Journal ArticleDOI
Idiopathic pulmonary fibrosis
TL;DR: This disease has improved understanding of the mechanisms of lung fibrosis, and offers hope that similar approaches will transform the management of patients with other progressive fibrotic lung diseases.
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Getting to the Heart of the Matter: New Insights Into Cardiac Fibrosis
TL;DR: This review addresses the contribution of a complex signaling network involving transforming growth factor-β, endothelin-1, angiotensin II, CCN2 (connective tissue growth factor), and platelet-derived growth factor to cardiac fibrosis.
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Effect of pirfenidone on proliferation, TGF-β-induced myofibroblast differentiation and fibrogenic activity of primary human lung fibroblasts.
TL;DR: It is demonstrated that pirfenidone modulates HLF proliferation and TGF-β-mediated differentiation into myofibroblasts by attenuating key TGF -β-induced signaling pathways.
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Immune Mechanisms in Pulmonary Fibrosis
TL;DR: The role of immune cells (T cells, neutrophils, macrophages, and fibrocytes) and soluble mediators (cytokines and chemokines) involved in pulmonary fibrosis are discussed, pointing toward novel immune-based therapeutic strategies in the field.
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Developmental pathways in the pathogenesis of lung fibrosis.
Diptiman Chanda,Eva Otoupalova,Samuel R. Smith,Thomas Volckaert,Stijn De Langhe,Victor J. Thannickal +5 more
TL;DR: Current knowledge in the field is analyzed, gained from both basic and clinical research, to provide new insights into the disease process, and potential approaches to treatment of pulmonary fibrosis.
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