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Proposed model for IPF. Chronic lung injury promotes lung epithelial cell death while (myo)fibroblasts become activated as a result of fibrosis inducing growth factors and cytokines. The failure of re-epithelization and hyper-proliferation of (myo)fibroblasts relentlessly produce collagen rich extracellular matrix, which promotes the fibrotic process, leading to the progression of lung fibrosis.
