title: Pulmonary fibrosis, part II: state-of-the-art patient management.
abstract: While many pharmacologic therapies for the treatment of idiopathic pulmonary fibrosis (IPF) have been evaluated via randomized, placebo-controlled clinical trials (RCTs) conducted over the past two decades, most therapies have been shown to be ineffective or even potentially harmful. However, a number of recently completed RCTs have shown significant efficacy for pirfenidone and nintedanib for the treatment of IPF. Areas covered: This manuscript reviews recent advances in the management of IPF and other forms of fibrosing interstitial lung disease (ILD) with an emphasis on IPF. The material upon which this discussion is based was obtained from various published texts and manuscripts identified via literature searching (e.g. PubMed). Expert commentary: Anti-fibrotic drugs are now available for clinical use and perceived as standard-of-care therapies that have the potential to blunt disease progression for many patients with IPF. However, these agents do not necessarily stop disease progression or have a significant impact on mortality, and more effective pharmacologic therapies are needed for patients with IPF. Additionally, whether anti-fibrotic agents can be effective therapies for other forms of pulmonary fibrosis, which often have radiologic and histopathologic manifestations that mimic IPF, is being evaluated in a number of RCTs.
authors.affiliation: a Department of Medicine , University of Wisconsin School of Medicine and Public Health - Medicine , K4/910 Clinical Science Center 600 Highland Avenue Madison, Madison , WI , 53792-9988 , USA.
authors.forename: Keith C
authors.initials: KC
authors.lastname: Meyer
mesh_terms: D006801:Humans; D011658:Pulmonary Fibrosis / Q000628:therapy*
keywords: Pulmonary fibrosis; idiopathic interstitial pneumonia; idiopathic pulmonary fibrosis; interstitial lung disease; treatment; usual interstitial pneumonia