Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing lung disease characterized by worsening lung scarring and a usual interstitial pneumonia pattern. It is a rapidly progressive illness of unknown cause with subsequent scarring that leads to end-stage lung disease
Validation data
Assessment of bodyweight, tissue morphology, and cytokine expression reveals interstitial pneumonia and fibrotic features in bleomycin-induced mouse lungs.
Drug efficacy
Immunohistochemistry indicates that, following Pirfenidone (PFD) treatment, there is a noticeable reduction in the expression of fibroblast marker α-SMA (brown). H&E staining (whole slide scan) and Masson's staining demonstrate that PFD can significantly alleviate the overall interstitial pneumonia induced by BLM (characterized by neutrophil infiltration foci and pulmonary parenchymal changes) as well as fibrosis (Masson's blue). Tests of pulmonary function demonstrate that PFD significantly improves the lung function of BLM-treated mice.