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Pirfenidone in Idiopathic Pulmonary Fibrosis: Expert Panel Discussion on the Management of Drug-Related Adverse Events

Research output: Contribution to journalLiterature review

Ulrich Costabel, Elisabeth Bendstrup, Vincent Cottin, Pieter Dewint, Jim J. J. Egan, James Ferguson, Richard Groves, Per M. Hellstrom, Michael Kreuter, Toby M. Maher, Maria Molina-Molina, Klas Nordlind, Alexandre Sarafidis, Carlo Vancheri

Original languageEnglish
Pages (from-to)375-391
Number of pages17
Issue number4
PublishedApr 2014

King's Authors


Pirfenidone is currently the only approved therapy for idiopathic pulmonary fibrosis, following studies demonstrating that treatment reduces the decline in lung function and improves progression-free survival. Although generally well tolerated, a minority of patients discontinue therapy due to gastrointestinal and skin-related adverse events (AEs). This review summarizes recommendations based on existing guidelines, research evidence, and consensus opinions of expert authors, with the aim of providing practicing physicians with the specific clinical information needed to educate the patient and better manage pirfenidone-related AEs with continued pirfenidone treatment. The main recommendations to help prevent and/or mitigate gastrointestinal and skin-related AEs include taking pirfenidone during (or after) a meal, avoiding sun exposure, wearing protective clothing, and applying a broad-spectrum sunscreen with high ultraviolet (UV) A and UVB protection. These measures can help optimize AE management, which is key to maintaining patients on an optimal treatment dose.

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