Idiopathic pulmonary fibrosis (IPF) is a severe chronic lung disease causing irreversible dysfunction of the organ. It is characterized by an abnormal repair process, causing uncontrolled deposition of extracellular matrix (ECM) including collagen, excessive proliferation of fibroblasts, and destruction of the cellular architecture of the lung. Bleomycin-induced pulmonary fibrosis in laboratory animals is a very well-characterized model, which can be used to study pathomechanisms directly related to human pulmonary fibrosis.
The challenge involves single or repeated exposure to bleomycin using a three or four-week protocol. This exposure has been shown to reproduce hallmarks of human fibrosis, including interstitial lung fibrosis, multifocal alveolar emphysema, accumulation of pigment-laden macrophages, and chronic active inflammation.