Pharmacological treatment of idiopathic pulmonary fibrosis: from the past to the future

Abstract

During the past decade important progress has been made regarding the pathogenesis of idiopathic pulmonary fibrosis (IPF), which is the most devastating form of idiopathic interstitial pneumonia with a median survival of 3 years. The knowledge gained has been used to design multicentre, randomised, placebo-controlled trials in order to investigate agents with different mechanisms of action. Encouraging results have led to licensing of the first IPF-specific drug, pirfenidone. However, the road to successful treatment is still long. The main aim for the future should be the careful design of clinical trials, by choosing the most clinically meaningful end-point and keeping in mind that combination of various agents may be more effective. This approach has been used in the treatment of lung cancer with which IPF presents many similarities. @ERSpublications Progress toward the treatment of IPF and aims for future clinical trials

Pirfenidone: an update on clinical trial data and insights from everyday practice

“…Although many of these clinical trials failed to demonstrate a statistically significant treatment effect, as recently reviewed by COTTIN [4,5] and ANTONIOU et al [6], positive results from two pivotal phase III trials (the CAPACITY (Clinical Studies Assessing Pirfenidone in IPF: Research of Efficacy and Safety Outcomes) programme) and two supportive Japanese trials in patients with IPF led to pirfenidone becoming the first agent to be approved for the treatment of patients with mild-to-moderate IPF in the European Union in 2011 [7][8][9]. Pirfenidone is also approved in Canada, Japan, India, China, South Korea and Argentina.…”

Fibrocytes in pulmonary fibrosis: a brief synopsis

“…Patients with IPF suffer from worsening dyspnoea and cough and can experience various patterns of progression during the disease course, eventually resulting in irreversible lung damage and decline of lung function [5]. This process often leads to respiratory failure with a median survival of 3 years after diagnosis [6,7].…”

Idiopathic pulmonary fibrosis and sleep disorders: no longer strangers in the night

“…We have passed into a new era of the therapy of IPF, with two IPF-specific drugs [7,8,79,80], and a third issue has emerged in the quest for the ideal biomarker for IPF. Future studies need to focus on the search for biomarkers with the property of identifying who is going to respond or not to these drugs.…”