Pirfenidone
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Pirfenidone is an agent used for the treatment of idiopathic pulmonary fibrosis (IPF).
Pirfenidone is an agent used for the treatment of idiopathic pulmonary fibrosis (IPF).
The exact mechanism of action of pirfenidone is not fully understood. It is suggested that the antioxidant effects of pirfenidone contribute to its anti-inflammatory effects, leading to antifibrotic effects. Pirfenidone attenuates the production of transforming growth factor-β1 (TGF-β1), a key profibrotic and pro-inflammatory cytokine implicated in idiopathic pulmonary fibrosis (IPF). By suppressing TGF-β1, pirfenidone inhibits TGF-β1-induced differentiation of human lung fibroblasts into myofibroblasts, thereby preventing excess collagen synthesis and extracellular matrix production. Some evidence suggests that pirfenidone downregulates pro-inflammatory cytokines, including TNF-α, interleukin-1 (IL-1), IL-6, interferon-gamma (IFN-γ), and platelet-derived growth factor (PDGF). Animal models demonstrated that pirfenidone promotes the production of anti-inflammatory IL-10 and prevents the accumulation of various inflammatory cells, including lymphocytes, macrophages and neutrophils. In animal models, pirfenidone inhibited the influx of inflammatory cells and ameliorated bleomycin-induced pulmonary vascular permeability. Several in vitro studies show that pirfenidone mediates antioxidant actions by scavenging reactive oxygen species (ROS) and inhibiting lipid peroxidation, thereby reducing cellular injury in IPF.
Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF). In Canada and Europe, it is approved in adults only.
共 3 项相关疾病
每项发明一条,涵盖美国、欧洲、PCT 与中国的同族专利。