TY - JOUR T1 - Long-term clinical and real-world experience with pirfenidone in the treatment of idiopathic pulmonary fibrosis JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 58 LP - 64 DO - 10.1183/09059180.00011514 VL - 24 IS - 135 AU - Vincent Cottin AU - Toby Maher Y1 - 2015/03/01 UR - http://err.ersjournals.com/content/24/135/58.abstract N2 - 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.Patient education is critical to help IPF patients manage side-effects of pirfenidone and remain on treatment http://ow.ly/HaPak ER -