Introduction: Idiopathic Pulmonary Fibrosis (IPF) is a progressive and devastating lung disease, characterized by progressive lung scarring.
Areas covered: Prior to antifibrotic therapy (pirfenidone and nintedanib), there was no validated pharmaceutical therapy for IPF. Both antifibrotics can slow disease progression; however, IPF remains a detrimental disease with poor prognosis and treatment survival rates of less than 7 years from diagnosis. Despite their effect the disease remains non-reversible and progressing whilst their side effect profile is often challenging. Treatment of comorbidities is also crucial. In this review, we discuss the current pharmacological management as well as management of comorbidities and symptoms. We also reviewed clinicaltrials.gov and summarized all the mid- to late-stage clinical trials (phase II and III) registered in IPF over the last 7 years and discuss the most promising drugs in clinical development.
Expert opinion: Future for IPF management will need to focus on current unresolved issues. First a primary pathogenetic pathway has not been clearly identified. Future management may involve a combination of the brushstroke approach with antifibrotics with targeted treatments for specific pathways in patient subsets following an 'oncological' approach. Another unmet need is the management of exacerbations, which are deadly in most cases, as well as either treating or preventing lung cancer.
Keywords: IPF; IPF management; IPF trials; drugs; emerging treatments; novel agents.