The pathogenesis idiopathic pulmonary of fibrosis and related fibrosis lung disorders are complex and poorly understood. This likely involves cellular mechanisms that result in loss of cellular homeostasis leading to aberrant alveolar wall remodeling through the excessive deposition of connective tissue matrices. Impaired tissue regeneration and dysregulation of cell death in lung fibroblasts and epithelial cells appear to be important in the initiation and progression of these disorders. This review summarizes current understanding in this area to stimulate research into the development of novel therapeutic strategies that prevent, halt or reverse the progression of lung fibrosis.