Pulmonary fibrosis, part II: state-of-the-art patient management

ABSTRACT

Introduction: 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.

KEYWORDS:

• Pulmonary fibrosis
• idiopathic pulmonary fibrosis
• idiopathic interstitial pneumonia
• usual interstitial pneumonia
• interstitial lung disease
• treatment

Declaration of interest

KC. Meyer has a partial sponsorship by a private entity (The George and Julie Mosher Pulmonary Research Fund. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Additional information

Funding

This paper was not funded.