From the Guidelines
The recommended treatment for idiopathic pulmonary fibrosis (IPF) includes antifibrotic medications such as pirfenidone (Esbriet) and nintedanib (Ofev), which slow disease progression by reducing lung function decline. These medications are typically taken daily - pirfenidone at 801 mg three times daily with food, and nintedanib at 150 mg twice daily with food 1.
Key Treatment Considerations
- Supportive care is equally important, including supplemental oxygen therapy when blood oxygen levels fall below 88%
- Pulmonary rehabilitation to improve exercise capacity and quality of life
- Vaccination against respiratory infections
- For eligible patients with advanced disease, lung transplantation remains the definitive treatment option
- Symptom management includes cough suppressants, pulmonary rehabilitation exercises, and treatment of gastroesophageal reflux which often coexists with IPF
Disease Management
These treatments work by either directly targeting the fibrotic process in the lungs or by managing symptoms and complications. Early diagnosis and treatment are crucial as these medications can slow but not reverse the fibrotic changes, and the disease has a progressive nature with median survival of 3-5 years without treatment. Patients should be evaluated and treated for existing comorbidities, including pulmonary hypertension, gastroesophageal reflux, obstructive sleep apnea, and lung cancer 1.
Ongoing Care
Patients may benefit from involvement of palliative care to help with symptom management (cough, dyspnea, and/or anxiety) 1. 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 1. Mechanical ventilation is not recommended for the majority of patients with respiratory failure.
From the FDA Drug Label
Pirfenidone is a pyridone indicated for the treatment of idiopathic pulmonary fibrosis (IPF). Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF). The recommended treatment for idiopathic pulmonary fibrosis (IPF) is pirfenidone.
- The recommended daily maintenance dosage of pirfenidone is 801 mg three times daily for a total of 2,403 mg/day.
- Doses should be taken with food at the same time each day.
- Upon initiation of treatment, titrate to the full dosage of 2,403 mg/day over a 14-day period 2, 2. Key points:
- Pirfenidone is used to treat IPF.
- The dosage should be titrated over 14 days to the full maintenance dosage.
- Patients should be monitored for adverse reactions and elevated liver enzymes.
From the Research
Treatment Options for Idiopathic Pulmonary Fibrosis (IPF)
The mainstay therapies for IPF are the oral antifibrotic drugs pirfenidone and nintedanib, which can improve quality of life, attenuate symptoms, and slow disease progression 3. These drugs have been shown to reduce the rate of decline in forced vital capacity and the risk of acute exacerbation among patients with IPF 4.
Recommended Treatment
- Monotherapy with pirfenidone or nintedanib is the mainstay of pharmacological treatment for IPF 5.
- Unilateral or bilateral lung transplantation is the only treatment for IPF shown to increase life expectancy 3.
- Combination of pirfenidone and nintedanib appears safe, but further research is needed to determine its efficacy 5.
Ongoing Research and Future Directions
Several clinical phase I-III trials are focusing on novel therapeutic targets, 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 5, 6. New approaches in nonpharmacological treatments, such as palliative care, pulmonary rehabilitation, lung transplantation, management of comorbidities, and acute exacerbations, aim to improve symptom control and quality of life 6.
Safety and Tolerability of Pirfenidone and Nintedanib
Real-world studies have evaluated the safety and tolerability profiles of pirfenidone and nintedanib in IPF patients, identifying potential predictors of treatment discontinuation or adverse drug reactions (ADRs) based on patient characteristics 7. The most common ADRs associated with nintedanib were gastrointestinal and hepatobiliary disorders, while pirfenidone was associated with skin, nervous system, and vascular disorders 7.