特发性肺纤维化人肺成纤维细胞

IPF (Idiopathic Pulmonary Fibrosis) is a fatal disease that usually affects adults between the ages of 50-70. The disease is characterized by progressive decline in lung function resulting from scarring of lung tissue. IPF varies from person to person where in some, fibrosis happens quickly, while the process can be much slower in others. IPF has no cure yet and both geneticand environmental factors are attributed to the development of the disease. Genetics may play a role in causing IPF, and cigarette smoking is the most accepted risk factor in increasing the chances of developing IPF. Recent research has helped doctors understand the disease more closely so they can more quickly diagnose it than in the past. Efforts are still underway in the research community to understand the pathways of disease progression and subsequently develop cures for IPF. IPF was once thought to be a chronic inflammatory process but recent evidence suggests that the abnormal activation of alveolar epithelial cells (AECs) induce over-activation of fibrotic response. The fibroblast and myofibroblast foci secrete excessive amounts of collagens, leading to scarringand destruction of the lung architecture. The mechanisms that link IPF with ageing and aberrant epithelial activation are unknown.

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