Summary

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• Esbriet (pirfenidone) is indicated for the treatment of idiopathic pulmonary fibrosis (IPF).
• This summary is based on the review of 13 systematic review(s)/meta-analysis(es). ^[1-13]
• Forced Vital Capacity (FVC) Improvement: Pirfenidone and Nintedanib have demonstrated efficacy in slowing FVC decline in IPF patients, with Pirfenidone showing a mean difference in predicted FVC of 2.3% and 100.0 ml in FVC. Pamrevlumab also showed improvement, with a 4.30% increase in predicted FVC and a 0.20L increase in absolute FVC.
• Disease Progression: Antifibrotic therapy (Pirfenidone and Nintedanib) reduced disease progression in fibrosing ILDs (RR = 0.56) and decreased all-cause mortality in progressive fibrosing ILDs (RR = 0.69).
• Mortality: Pirfenidone significantly reduced all-cause mortality in IPF patients (pooled RR = 0.51; OR = 0.50). Nintedanib also reduced mortality in progressive fibrosing ILDs and showed comparable efficacy to Pirfenidone in IPF.
• Acute Exacerbations: Both Pirfenidone and Nintedanib reduced the risk of acute exacerbations in IPF patients, with a pooled RR of 0.63.
• General Safety Outcomes: Pirfenidone commonly caused gastrointestinal symptoms (diarrhea, dyspepsia, vomiting), photosensitivity, and skin rashes. Nintedanib primarily led to gastrointestinal issues such as diarrhea and nausea.
• Serious Adverse Events (SAEs): Pirfenidone had a higher incidence of adverse events with a pooled relative ratio of 3.89, including an increased risk of gastrointestinal discomfort (RR 1.83) and photosensitivity (RR 4.88). Combination therapy with Pirfenidone and Nintedanib resulted in frequent serious adverse drug reactions (ADRs), with 10% of patients experiencing serious ADRs.
• Discontinuation Rates: High discontinuation rates were observed with combination therapy, with 29% of patients discontinuing due to adverse effects.
• Antifibrotic therapy, including Pirfenidone and Nintedanib, was effective in reducing disease progression and mortality in patients with progressive fibrosing ILDs, and both drugs demonstrated a significant reduction in FVC decline in patients with RA-ILD, with more pronounced gastrointestinal side effects and photosensitivity noted in specific populations such as those with high sun exposure.