The concept on idiopathic pulmonary fibrosis (IPF) pathogenesis has progressed from chronic inflammation to aberrant wounding healing and even more to the current paradigms of a multifactorial and heterogeneous disease process. Despite the growth of clinical trials for IPF, most of the results, including N-acetylcysteine combination, warfarin, and bosentan, were disappointing. On the other hand, there have been a number of important developments; the foremost is the licensing of pirfenidone in Europe and Asia. In this article, we briefly review the recent knowledge of pathogenesis of IPF. We also summarize the recent clinical trials regarding the management of IPF. Copyright
Bibliographical noteFunding Information:
This research was supported in part by National Institutes of Health, National Heart, Lung, and Blood Institute grants P50HL-56402 and 2 K24 HL04212.
- Clinical trial
- Idiopathic pulmonary fibrosis
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
- Infectious Diseases