From the Guidelines
Pulmonary fibrosis patients should be treated with antifibrotic medications such as nintedanib or pirfenidone, and considered for lung transplantation if they meet specific criteria, as recommended by the most recent clinical practice guideline 1. Pulmonary fibrosis is a chronic lung disease characterized by progressive scarring of lung tissue, leading to breathing difficulties.
Treatment Options
- Antifibrotic medications such as nintedanib (Ofev) at 150 mg twice daily or pirfenidone (Esbriet) at 801 mg three times daily can slow disease progression but not reverse existing damage.
- Supplemental oxygen therapy is recommended when blood oxygen levels fall below 88%, starting at 2-4 liters per minute and adjusted based on needs.
- Pulmonary rehabilitation programs combining exercise training, breathing techniques, and education are essential for symptom management.
Disease Management
- Patients should be evaluated and treated for existing comorbidities, including pulmonary hypertension, gastroesophageal reflux, obstructive sleep apnea, and lung cancer.
- Patients may benefit from involvement of palliative care to help with symptom management (cough, dyspnea, and/or anxiety).
- Patient values and preferences should be explored.
- Patients at increased risk of mortality should be referred for lung transplantation at diagnosis.
- Patients should be evaluated every 3–6 months or more often for disease progression.
- Acute exacerbations may be treated with corticosteroids, however, mechanical ventilation is not recommended for the majority of patients with respiratory failure 1. The disease results from repeated injury to alveolar epithelial cells, triggering abnormal wound healing processes where fibroblasts produce excessive collagen and extracellular matrix, causing the characteristic scarring.
Risk Factors and Monitoring
- Risk factors include smoking, environmental exposures, certain medications, and genetic predisposition.
- Regular monitoring with pulmonary function tests every 3-6 months is necessary to track disease progression and adjust treatment accordingly, as supported by previous studies 1.
From the FDA Drug Label
Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF). Pirfenidone is used to treat idiopathic pulmonary fibrosis (IPF).
- The primary endpoint in clinical studies was the change in percent predicted forced vital capacity (%FVC) from baseline to study end.
- Key findings include a reduction in the mean decline in FVC (in mL) in patients receiving pirfenidone compared to placebo 2.
- Survival was evaluated, but all-cause mortality did not show a statistically significant difference between pirfenidone and placebo 2.
From the Research
Definition and Overview of Pulmonary Fibrosis
- Pulmonary fibrosis is a progressive and ultimately fatal lung disease associated with dyspnoea, cough, and impaired quality of life 3.
- It is characterized by progressive scarring of the lung and associated with a high burden of disease and early death 4.
Treatment and Management of Pulmonary Fibrosis
- The aims of patient care are to improve outcomes for patients by slowing the progression of the disease, extending life, and improving quality of life 3.
- Two anti-fibrotic drugs, nintedanib and pirfenidone, have been shown to reduce decline in lung function in patients with IPF 3, 5, 6.
- Optimal management of IPF includes treatment of comorbidities, symptom relief, pulmonary rehabilitation, and palliative care 3, 7.
- Patient education is important to enable patients to make decisions about their care and to help them manage their disease and the side-effects of anti-fibrotic drugs 3.
Efficacy of Pirfenidone and Nintedanib
- Pirfenidone and nintedanib have been shown to reduce the rate of decline in forced vital capacity and the risk of acute exacerbation among patients with idiopathic pulmonary fibrosis 5, 6.
- Nintedanib has been demonstrated to be effective in slowing disease progression in patients with interstitial lung diseases other than idiopathic pulmonary fibrosis 5.
- Results on the use of pirfenidone in interstitial lung diseases other than idiopathic pulmonary fibrosis should be interpreted with more caution due to trial limitations 5.
Future Directions and Ongoing Research
- Research continues into new treatments and combinations of treatments that may improve outcomes for patients with pulmonary fibrosis 3, 4, 7.
- Several clinical phase I-III trials are focusing on novel therapeutic targets, and new approaches in nonpharmacological treatments aim to improve symptom control and quality of life 4, 7.