From the Guidelines
Treatment options for pulmonary fibrosis should include antifibrotic medications such as pirfenidone and nintedanib, which can slow disease progression, as well as nonpharmacological therapies like oxygen supplementation and pulmonary rehabilitation, according to the most recent guidelines 1. The goal of treatment is to preserve lung function, reduce symptoms, and improve quality of life.
- Antifibrotic medications:
- Pirfenidone: typically initiated at 267 mg three times daily and gradually increased to 801 mg three times daily over two weeks
- Nintedanib: typically dosed at 150 mg twice daily
- Nonpharmacological therapies:
- Oxygen supplementation: recommended when oxygen saturation falls below 88%
- Pulmonary rehabilitation: can help improve exercise capacity and quality of life
- Other considerations:
- Corticosteroids and immunosuppressants may be used for certain types of pulmonary fibrosis with inflammatory components
- Lung transplantation may be considered in eligible patients with end-stage disease
- Comorbidities like gastroesophageal reflux disease should be addressed, as they can worsen fibrosis
- Palliative care may be beneficial for symptom management, including cough, dyspnea, and anxiety 1 It is essential to evaluate and treat existing comorbidities, such as pulmonary hypertension, gastroesophageal reflux, obstructive sleep apnea, and lung cancer, and to consider patient values and preferences when developing a treatment plan 1. Regular follow-up, every 3-6 months, is crucial to monitor disease progression and adjust treatment accordingly 1. In cases of acute exacerbations, corticosteroids may be used, but mechanical ventilation is not recommended for the majority of patients with respiratory failure 1. The most recent guidelines suggest that nintedanib may be considered for the treatment of progressive pulmonary fibrosis in patients who have failed standard management for fibrotic ILD, other than IPF 1. However, further research is needed to determine the efficacy, effectiveness, and safety of pirfenidone and nintedanib in specific types of non-IPF ILD manifesting progressive pulmonary fibrosis 1.
From the FDA Drug Label
1 INDICATIONS AND USAGE
Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF).
The recommended treatment option for patients with pulmonary fibrosis is pirfenidone.
- Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF) 2.
- The primary endpoint was the change in percent predicted forced vital capacity (%FVC) from baseline to study end, measured at 52 weeks in Study 1, and at 72 weeks in Studies 2 and 3 2.
- Pirfenidone has been shown to reduce the decline in FVC volume in patients with IPF 2.
- Key benefits of pirfenidone include:
- Reduction in decline of FVC
- Slow down of disease progression
- Improvement of quality of life
From the Research
Treatment Options for Pulmonary Fibrosis
From a respiratory therapist's perspective, treatment options for patients with pulmonary fibrosis include:
- Antifibrotic medications, such as nintedanib and pirfenidone, which have been shown to slow the rate of decline in lung function 3, 4, 5
- Comprehensive care, including a multidisciplinary evaluation, diagnosis and treatment of comorbid medical conditions, and referral to and participation in a pulmonary rehabilitation program 3
- Switching antifibrotic therapy may be a feasible management strategy for patients who do not tolerate first-line antifibrotic treatment or those showing disease progression despite treatment 4
Antifibrotic Medications
The two main antifibrotic medications used to treat pulmonary fibrosis are:
- Nintedanib, which may slow the decline in health-related quality of life for treated patients 3
- Pirfenidone, which may slow the progression of dyspnea and improve cough 3
Considerations for Treatment
When starting antifibrotic therapy, patients and providers should participate in shared decision-making, taking into consideration the benefits of treatment in addition to drug-related side effects and dosing schedules 3 The discontinuation rate due to adverse events within one year of antifibrotic treatment was significantly higher in the nintedanib group than in the pirfenidone group 6
Future Directions
Novel therapies, including the autotaxin-lysophosphatidic acid (ATX/LPA) pathway, connective tissue growth factor (CTGF), pentraxin-2, G protein-coupled receptor agonists/antagonists, αvβ6 integrin, and galectin-3, hold great promise for the future treatment of pulmonary fibrosis 7