From the Guidelines
The FDA has approved two drugs specifically for the treatment of interstitial lung disease (ILD): nintedanib (Ofev) and pirfenidone (Esbriet). These medications are primarily approved for idiopathic pulmonary fibrosis (IPF), which is a specific type of ILD. Nintedanib is also approved for ILD associated with systemic sclerosis and chronic fibrosing ILDs with a progressive phenotype 1.
Key Points
- The typical dosage for nintedanib is 150 mg twice daily, while pirfenidone is usually taken as 801 mg three times daily (after a titration period) 1.
- Both medications work by slowing disease progression rather than curing the condition.
- Nintedanib functions as a tyrosine kinase inhibitor that blocks growth factor receptors involved in pulmonary fibrosis, while pirfenidone has anti-inflammatory and anti-fibrotic properties 1.
- Common side effects of nintedanib include diarrhea and liver enzyme elevations, while pirfenidone commonly causes gastrointestinal issues, rash, and photosensitivity.
- Regular monitoring of liver function is required for both medications 1.
- Treatment decisions should be individualized based on the specific type of ILD, disease severity, and patient comorbidities 1.
Recommendations
- For people with SSc-ILD, nintedanib is conditionally recommended as a first-line ILD treatment option 1.
- For people with SARD-ILD other than SSc-ILD, glucocorticoids are conditionally recommended as a first-line ILD treatment 1.
- For people with IIM-ILD, JAKi is conditionally recommended as a first-line ILD treatment option 1.
From the FDA Drug Label
The efficacy of pirfenidone was evaluated in patients with IPF in three phase 3, randomized, double-blind, placebo-controlled, multicenter trials (Studies 1,2, and 3). Study 1 was a 52-week trial comparing pirfenidone 2,403 mg/day (n=278) versus placebo (n=277) in patients with IPF Study 2 and Study 3 were nearly identical to each other in design, with few exceptions, including an intermediate dose treatment arm in Study 2. Study 2 compared treatment with either pirfenidone 2,403 mg/day (n=174) or pirfenidone 1,197 mg/day (n=87) to placebo (n=174), while Study 3 compared pirfenidone 2,403 mg/day (n=171) to placebo (n=173)
Pirfenidone is an FDA-approved drug for the treatment of Idiopathic Pulmonary Fibrosis (IPF), a type of Interstitial Lung Disease (ILD) 2.
- The primary endpoint was the change in percent predicted forced vital capacity (%FVC) from baseline to study end.
- Key findings include a statistically significant treatment effect of pirfenidone in Study 1 and Study 2, but not in Study 3.
- Pirfenidone is administered orally, with a recommended dose of 2,403 mg/day.
From the Research
FDA Approved Drugs for ILD
- The US Food and Drug Administration (FDA) has approved two antifibrotic agents, pirfenidone and nintedanib, for the treatment of idiopathic pulmonary fibrosis (IPF) 3.
- These drugs have been shown to reduce the rate of decline in forced vital capacity (FVC) among patients with IPF 4, 3.
- Pirfenidone and nintedanib may also be efficacious in non-IPF progressive fibrotic interstitial lung diseases (non-IPF-PF) due to similar pathogenetic and biological pathways 4, 5.
- A systematic review summarized data on the efficacy of pirfenidone and nintedanib in interstitial lung diseases other than IPF, with results suggesting that nintedanib can slow disease progression in these patients 5.
Treatment Options for ILD
- Antifibrotic therapy with nintedanib or pirfenidone slows annual FVC decline by approximately 44% to 57% in individuals with IPF, scleroderma-associated ILD, and in those with progressive pulmonary fibrosis of any cause 6.
- Immunomodulatory therapy, such as tocilizumab, rituximab, and mycophenolate mofetil, may slow decline or even improve FVC at 12-month follow-up in patients with connective tissue disease-associated ILD 6.
- First-line therapy for ILD includes nintedanib or pirfenidone for IPF and mycophenolate mofetil for ILD due to connective tissue disease 6.