Summary

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• This summary is based on the review of 13 systematic review(s)/meta-analysis(es). ^[1-13]
• Pirfenidone vs. Placebo: In idiopathic pulmonary fibrosis (IPF) patients, pirfenidone significantly slowed the decline in lung function, as measured by forced vital capacity (FVC), improved progression-free survival, and reduced acute exacerbations, showing superior efficacy to placebo in multiple studies.
• Pirfenidone vs. Nintedanib: Both pirfenidone and nintedanib were effective in reducing the rate of FVC decline in IPF and showed similar efficacy in non-IPF fibrosing interstitial lung diseases (ILDs), with no significant differences between the two in disease progression outcomes.
• Combination Therapy (Pirfenidone + Nintedanib): Data on combined use of pirfenidone and nintedanib suggest frequent adverse reactions and high discontinuation rates, with limited evidence supporting additional efficacy over monotherapy in IPF.
• Non-IPF Fibrosing ILDs: Pirfenidone demonstrated efficacy in slowing disease progression in non-IPF fibrosing ILDs, with comparable outcomes to those observed in IPF, including similar effects on FVC decline.
• Pirfenidone vs. Placebo: Pirfenidone treatment in IPF patients was associated with a higher incidence of adverse events compared to placebo, particularly gastrointestinal symptoms (diarrhea, dyspepsia, vomiting), photosensitivity, and skin rashes, but the treatment’s benefits in slowing disease progression were maintained despite these side effects.
• Pirfenidone vs. Nintedanib: Both pirfenidone and nintedanib were associated with similar gastrointestinal side effects, though photosensitivity and skin-related adverse events were more frequent with pirfenidone.
• Combination Therapy (Pirfenidone + Nintedanib): Combination therapy with pirfenidone and nintedanib had a high incidence of adverse drug reactions (82%) and resulted in treatment discontinuation in 29% of cases, with gastrointestinal symptoms and acute exacerbations of IPF as the primary adverse reactions.
• There is no population types or subgroups information available in the reviewed studies.