Die idiopathische Lungenfibrose

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF

Effects and mechanisms of pirfenidone, prednisone and acetylcysteine on pulmonary fibrosis in rat idiopathic pulmonary fibrosis models

“…Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and characterized by extracellular matrix (ECM) remodelling and abnormal proliferation of fibroblasts in pulmonary parenchyma (Thannickal et al. 2004 ; Prasse 2015 ). IPF results from long-term exposure to substances harming alveolar epithelial cells, and also heredity (Prasse 2015 ).…”

“… 2004 ; Prasse 2015 ). IPF results from long-term exposure to substances harming alveolar epithelial cells, and also heredity (Prasse 2015 ). Currently, the pathogenesis of pulmonary fibrosis has not been clearly uncovered, but various cytokines and other molecules have been discovered to play crucial roles in the progression of pulmonary fibrosis, such as tumour necrosis factor-α (TNF-α) (Pantelidis et al.…”