From the Guidelines
The treatment for pulmonary fibrosis should prioritize antifibrotic medications, such as nintedanib and pirfenidone, alongside nonpharmacological therapies like oxygen supplementation and pulmonary rehabilitation, to improve morbidity, mortality, and quality of life. According to the most recent guideline evidence 1, treatment considerations should include both pharmacological and nonpharmacological therapies. Patients should be evaluated and treated for existing comorbidities, including pulmonary hypertension, gastroesophageal reflux, obstructive sleep apnea, and lung cancer.
Key Treatment Components
- Antifibrotic medications: nintedanib (Ofev) at 150 mg twice daily or pirfenidone (Esbriet) at 801 mg three times daily, as they can slow disease progression by reducing scarring in the lungs 1.
- Oxygen therapy: supplemental oxygen is recommended when blood oxygen levels fall below 88% to improve exercise capacity and quality of life.
- Pulmonary rehabilitation: programs help improve exercise capacity and quality of life, with beneficial effects more pronounced in patients with worse baseline functional status 1.
- Gastroesophageal reflux disease (GERD) treatment: proton pump inhibitors like omeprazole (20-40 mg daily) to aggressively treat reflux, as it can worsen fibrosis.
- Vaccination: against influenza and pneumococcal pneumonia to prevent respiratory infections.
Additional Considerations
- Patient values and preferences should be explored, and patients may benefit from involvement of palliative care to help with symptom management (cough, dyspnea, and/or anxiety) 1.
- Patients at increased risk of mortality should be referred for lung transplantation at diagnosis, as it remains the definitive treatment for advanced disease in suitable candidates.
- Regular evaluation every 3–6 months or more often for disease progression is crucial, and acute exacerbations may be treated with corticosteroids 1.
From the FDA Drug Label
Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF). The recommended daily maintenance dosage of pirfenidone is 801 mg three times daily for a total of 2,403 mg/day.
- Treatment: Pirfenidone is used for the treatment of idiopathic pulmonary fibrosis (IPF).
- Dosage: The recommended daily dosage is 801 mg three times daily, for a total of 2,403 mg/day. 2
From the Research
Treatment Options for Pulmonary Fibrosis
- Pirfenidone and nintedanib are two medications that have been approved for the treatment of idiopathic pulmonary fibrosis (IPF) and can slow disease progression 3, 4.
- Pirfenidone has been shown to inhibit the decline of forced vital capacity (FVC)/vital capacity (VC) and may decrease the mortality rate 5.
- A systematic review and meta-analysis found that pirfenidone was associated with reduced declines in vital capacity (VC) and forced vital capacity (FVC) from baseline in patients with IPF, and was also associated with lower reductions in FVC, lower reductions in 6-minute walking test distance, and lower all-cause death 6.
- Nintedanib has also been shown to slow disease progression and improve outcomes in patients with IPF 3, 4.
- Pulmonary rehabilitation remains a critical part of management and has been shown to improve quality of life and functional level 3.
- Lung transplantation is also an option for some patients with IPF, although it is typically reserved for those with advanced disease 3.
Management of Acute Exacerbations
- Acute exacerbations of IPF (AE-IPF) can be life-threatening and require hospitalization 7.
- Innovative pharmacologic treatments and supportive therapeutic strategies have been shown to prolong survival and reduce the risk of in-hospital mortality in patients with AE-IPF hospitalized for acute respiratory failure (ARF) 7.
- A study found that survival time was significantly longer in patients who received newer treatments for AE-IPF compared to those who received older treatments 7.
Safety and Efficacy of Pirfenidone
- Pirfenidone has been shown to be effective in delaying the progression of IPF, but it can also cause adverse reactions such as gastrointestinal, skin, nervous system, and liver function-related events 6.
- The incidence of these adverse events was significantly higher in the pirfenidone group compared to the control group, but pirfenidone was still considered to be well-tolerated by the majority of patients 6.