From the Guidelines
The treatment of idiopathic pulmonary fibrosis (IPF) should focus on slowing disease progression, managing symptoms, and improving quality of life, with the use of antifibrotic medications such as pirfenidone and nintedanib, as recommended by the most recent guidelines 1.
Treatment Approach
The treatment approach for IPF should be individualized based on disease severity, comorbidities, medication tolerability, and patient preferences.
- Two antifibrotic medications are FDA-approved for IPF: pirfenidone (Esbriet) at 801 mg three times daily and nintedanib (Ofev) at 150 mg twice daily, which slow lung function decline but don't reverse existing fibrosis 1.
- Supportive care is essential and includes supplemental oxygen when oxygen saturation falls below 88%, pulmonary rehabilitation to improve exercise capacity and quality of life, and vaccination against influenza, pneumococcal pneumonia, and COVID-19.
- Symptom management includes cough suppressants like codeine (15-30 mg every 4-6 hours) or benzonatate (100-200 mg three times daily), and treatment of gastroesophageal reflux with proton pump inhibitors.
Disease Management
- Lung transplantation should be considered for appropriate candidates with advanced disease.
- Corticosteroids and immunosuppressants are not recommended for IPF as they may worsen outcomes, as stated in the most recent guidelines 1, which contradicts older studies 1.
- Early referral to a specialized interstitial lung disease center is important for accurate diagnosis, optimal treatment planning, and potential clinical trial participation.
- Patients should be evaluated every 3–6 months or more often for disease progression, and acute exacerbations may be treated with corticosteroids, as shown in Figure 11 of the guideline 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 of IPF: Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF) 2. The recommended dosage is 801 mg three times daily, with a total daily dose of 2,403 mg/day.
- Dosage: The daily maintenance dosage of pirfenidone is 801 mg three times daily for a total of 2,403 mg/day.
- Administration: Doses should be taken with food at the same time each day.
- Titration: Upon initiation of treatment, titrate to the full dosage of 2,403 mg/day over a 14-day period. 2
From the Research
Treatment Options for 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, 4, 5, 6, 7.
- Unilateral or bilateral lung transplantation is the only treatment for IPF shown to increase life expectancy 3.
- Other approaches, such as palliative care, pulmonary rehabilitation, management of comorbidities, and acute exacerbations, aim to improve symptom control and quality of life 4.
Antifibrotic Therapies
- Pirfenidone and nintedanib have been extensively validated as antifibrotic drugs, but some patients reported side effects, including gastrointestinal symptoms, photosensitivity, and skin rashes 6.
- Dose adjustment has been shown to be effective at reducing side effects without compromising efficacy 7.
- Antifibrotic therapies slow decline in lung function and reduce the risk of acute respiratory deteriorations, which are associated with high morbidity and mortality 7.
Future Directions
- There is an unmet need for accelerated research into IPF mechanisms to develop new therapeutic approaches 3, 4, 5.
- Several clinical trials are currently being conducted to evaluate novel therapeutic targets and developmental molecules 4, 5.
- Further research is needed to optimize the use of antifibrotic agents in patients with IPF and to establish treatments that can stop disease progression 6.