Idiopathic pulmonary fibrosis--a disorder of alveolar wound repair?

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic and usually progressive lung disorder of unknown aetiology. Conventional management of patients with IPF has been primarily based on the concept that suppressing inflammation would prevent progression to fibrosis. Although the pathogenesis is incompletely understood, it is here suggested that IPF is a disease of abnormal wound repair and remodelling in the lung rather than an inflammatory disease. Therefore, treatment strategies are no longer aimed at reducing inflammation, but rather at preventing or inhibiting the fibroproliferative responses and enhancing efficient alveolar epithelial repair. So far, no cell-specific drugs for these purposes are clinically available. However, novel promising molecules or drugs are being studied in experimental models or ongoing clinical trials in patients with IPF. Evolving hypotheses on the pathogenesis of IPF are reviewed, focusing on possible implications for future therapies. A better understanding of the sequence of the pathogenic mechanisms that control the fibrotic response will hopefully lead to efficient therapies and finally a favourable outcome in patients with this disease.