What are the highlights of Idiopathic (Idiopathic) Pulmonary Fibrosis (IPF)?

From the Guidelines

Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible lung disease characterized by scarring of lung tissue, leading to declining lung function and respiratory failure, and its management should prioritize antifibrotic therapies, supportive care, and consideration of lung transplantation for advanced disease, as recommended by the most recent guidelines 1.

Key Highlights

• Typical presentation in older adults (60-70 years) with symptoms of progressive dyspnea, dry cough, and fine inspiratory crackles on examination
• Diagnosis requires high-resolution CT showing a usual interstitial pneumonia pattern and exclusion of other causes of interstitial lung disease
• Treatment options include antifibrotic medications like pirfenidone and nintedanib, which slow disease progression but don't reverse existing damage

Treatment Approach

• Antifibrotic medications, such as pirfenidone (801 mg three times daily) and nintedanib (150 mg twice daily), are recommended as first-line treatment for IPF, as they have been shown to slow disease progression 1
• Supportive care is essential, including supplemental oxygen when hypoxemia develops, pulmonary rehabilitation, vaccination against respiratory infections, and management of comorbidities
• Lung transplantation should be considered for appropriate candidates with advanced disease

Monitoring and Prognosis

• Regular monitoring of pulmonary function tests every 3-6 months is recommended to assess disease progression and treatment response
• Prognosis is generally poor with median survival of 3-5 years from diagnosis, though antifibrotic therapy has improved outcomes, as noted in the most recent guidelines 1

Clinical Considerations

• Patient values and preferences should be explored, and palliative care should be considered to help with symptom management (cough, dyspnea, and/or anxiety) 1
• Acute exacerbations may be treated with corticosteroids, and mechanical ventilation is not recommended for the majority of patients with respiratory failure 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). In all three trials, over 80% of patients completed study treatment A total of 1,247 patients with IPF were randomized to receive pirfenidone 2,403 mg/day (n=623) or placebo (n=624) in these three trials. Change from Baseline in Percent Predicted Forced Vital Capacity In Study 1, the primary efficacy analysis for the change in %FVC from baseline to Week 52 demonstrated a statistically significant treatment effect of pirfenidone 2,403 mg/day (n=278) compared with placebo (n=277) For all categorical declines in lung function, the proportion of patients declining was lower on pirfenidone than on placebo.

The main highlights of pirfenidone treatment for idiopathic pulmonary fibrosis (IPF) are:

• Improved lung function: Pirfenidone treatment resulted in a statistically significant reduction in the decline of forced vital capacity (FVC) compared to placebo in Study 1 and Study 2.
• Reduced decline in lung function: The proportion of patients with categorical declines in lung function was lower on pirfenidone than on placebo.
• No significant difference in mortality: All-cause mortality did not show a statistically significant difference between pirfenidone and placebo treatment groups 2.

From the Research

Admilparent Pulmonary Fibrosis Highlights

• There are no specific studies on "Admilparent pulmonary fibrosis" in the provided evidence, however, the term is likely a misspelling or variation of "idiopathic pulmonary fibrosis" (IPF) or other forms of pulmonary fibrosis.
• The provided studies discuss the treatment and management of IPF and other forms of pulmonary fibrosis, including the use of antifibrotic medications such as pirfenidone and nintedanib 3, 4, 5, 6, 7.
• These medications have been shown to slow the decline in lung function and improve quality of life for patients with IPF 3, 4, 6.
• The studies also discuss the potential use of these medications in non-IPF forms of pulmonary fibrosis, as well as the need for further research into the treatment and management of these conditions 3, 5, 7.
• Common side effects of pirfenidone and nintedanib include gastrointestinal symptoms, photosensitivity, and skin rashes 5, 6.
• Combination therapy with pirfenidone and nintedanib has been shown to be safe and effective in some patients with IPF, but may increase the risk of certain side effects 6.
• Lung transplantation is currently the only treatment that has been shown to increase life expectancy for patients with IPF 7.