Treatment
In This Section
Treatment pathways
In this section, learn about the current advice on the management of patients with idiopathic pulmonary fibrosis (IPF), including patient monitoring and interventions, as well therapies in development.
Monitoring the clinical course of disease
Monitoring patients with idiopathic pulmonary fibrosis (IPF) is important to identify patients with disease progression and/or potential treatment complications.2
Monitoring should be done every four to six months or sooner as clinically indicated.2
In the absence of another identifiable cause, the presence of any of the following indicates progressive disease:2
- progressive dyspnoea (objectively assessed)
- progressive, sustained decrease from baseline in absolute forced vital capacity
- progressive, sustained decrease from baseline in absolute DLCO (corrected for haemoglobin)
- progression of fibrosis from baseline on high-resolution computed tomography
- acute exacerbation
- death from respiratory failure
Pulmonary function testing provides the most standardised approach to monitoring and quantification of disease progression.2
Interventions
In this section about interventions for patients with idiopathic pulmonary fibrosis (IPF), find out about current therapies, including their mode of action and side effects, non-pharmaceutical interventions and the importance of early treatment and managing exacerbations.
Therapies
In the absence of a cure for idiopathic pulmonary fibrosis (IPF), the goals of patient care are:23
- to slow the progression of the disease
- preserve quality of life
- improve survival
Nintedanib and pirfenidone
Two anti-fibrotic drugs, nintedanib and pirfenidone, have both proven to be effective in significantly reducing lung functional decline in patients with IPF compared to placebo (see Figures 8 and 9).24,25
Figure 8. Adjusted annual rate of change in forced vital capacity (FVC) in two randomised, double-blind, phase III clinical trials (and open-label extension) of nintedanib in patients with IPF.25,26
Figure 9. Percentage of patients with a meaningful decrease in % FVC (change ≥10% decline) at Week 52 in a phase III clinical trial of pirfenidone in patients with IPF.24
Nintedanib and pirfenidone have also been shown to potentially reduce mortality in patients with IPF.27,28 In a prespecified pooled analysis of clinical trials in patients with IPF.27,28
- pirfenidone compared with placebo significantly reduced all-cause mortality (3.5% versus 6.7%, respectively; P=0.01, hazard ratio (HR): 0.52)
- nintedanib compared with placebo numerically reduced all-cause mortality (5.5% versus 7.8%, respectively; P=0.14, HR: 0.70)
The US FDA approved both pirfenidone and nintedanib in 2014 with no prescription limitations and with no restrictions to lung function impairment severity.10 However, so far clinical trials have been limited to patients with mild-to-moderate IPF and more information on effectiveness in patients with IPF with greater impairment are needed.13
Expert-led assessment of relevant clinical guidance
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Guidelines
The 2015 international guidelines on treatment of idiopathic pulmonary fibrosis (IPF) by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society and Latin American Thoracic Association recommend two anti-fibrotic treatments and anti-acid therapy for patients with IPF (Table 6).13
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IPF References
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