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Competing Interests: Non-conditional grants von Boehringer-Ingelheim, Schweiz; Non-conditional grants von Roche Pharma, Schweiz; Speaker-fees von Boehringer-Ingelheim, Schweiz., (© 2024 Aerzteverlag medinfo AG.)

Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonias (IIP). It is characterized by progressive destruction of the normal lung architecture, finally leading to death. The pathogenesis of IPF is not completely understood but a complex interplay between environmental factors, such as smoking or viral infections, and genetic predisposition seems to be an important precondition. Repetitive micro-injuries to alveolar epithelial cells and dysregulated wound repair with impaired re-epithelialization are regarded as the initial process of IPF. This alveolar damage is followed by an uncontrolled proliferation and extracellular matrix deposition of fibroblasts, ultimately leading to distortion of the lung parenchyma. According to this "non-inflammatory" pathogenic hypothesis, therapeutic approaches currently focus on anti-fibrotic compounds, which prevent or inhibit fibroblast proliferation and extracellular matrix deposition, or antagonize the effect of pro-fibrotic growth factors.