Long-term clinical and real-world experience with pirfenidone in the treatment of idiopathic pulmonary fibrosis.
Idiopathic pulmonary fibrosis (IPF) is a chronic, irreversible, progressively destructive lung disease that culminates in respiratory failure and death. Randomised controlled trials have demonstrated that treatment of IPF patients with pirfenidone reduces lung function decline, improves progression-free survival and significantly reduces the risk of all-cause mortality at 1 year. Pirfenidone has been shown to have a favourable safety profile and was generally well tolerated over the long term in clinical trials and real-world experience. However, side-effect management is critical to help some patients remain on treatment over the long term. The primary treatment-related adverse events associated with pirfenidone therapy are gastrointestinal upset, rash and photosensitivity. Gastrointestinal events may be mitigated by ensuring that pirfenidone is taken with food, while skin symptoms may be reduced by avoiding sun exposure and frequent use of sunblock. Educating patients about the potential for these adverse events to occur and providing instructions prior to treatment to avoid adverse drug reactions are an important means of ensuring patients may derive the important benefits provided by long-term treatment with pirfenidone.
National Reference Centre for Rare Pulmonary Diseases, Dept of Respiratory Medicine, Louis Pradel Hospital, Claude Bernard Lyon 1 University, UMR 754, Lyon, France.
Vincent
V
Cottin
National Heart and Lung Institute, Imperial College, London, UK NIHR Respiratory Biomedical Research Unit, Royal Brompton Hospital, London, UK.
Toby
T
Maher
D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy*; D011728:Pyridones / Q000009:adverse effects / Q000627:therapeutic use*; D012042:Registries
D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone