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Antifibrotic therapy of Treatment of idiopathic pulmonary fibrosis
For patients with mild-to-moderate IPF based on pulmonary function tests who live in an area where either pirfenidone or nintedanib is available, we recommend initiating therapy with the available agent (Grade 1B). Current data are insufficient to direct a firm choice between nintedanib and pirfenidone. Patient preference and tolerances should be considered, particularly regarding potential adverse effects. (See ‘Our approach’ above and ‘Assessing disease severity and prognosis’ above.)
•The dose of nintedanib is 150 mg by mouth twice daily. Patients with known liver disease (Pugh B or worse) or full anticoagulation are not candidates for nintedanib. Diarrhea, nausea, vomiting, and liver function test elevation are common side effects of nintedanib. (See ‘Nintedanib’ above.)
•The dose of pirfenidone ranges up to 40 mg/kg per day (to maximum of 2403 mg per day) in three divided oral doses. Rash, photosensitivity, nausea, and abdominal discomfort are common side effects of pirfenidone. (See ‘Pirfenidone’ above.)
•In patients with more severe IPF, we also suggest therapy with either nintedanib or pirfenidone (Grade 2C). Based on observational data, nintedanib and pirfenidone appear to reduce lung function decline in patients with more advanced disease, like the effect in less advanced disease. The decision to try one of these medications depends on the values and preferences of the patient regarding a choice of active therapy with substantial adverse effects versus supportive care alone. (See ‘Role in more advanced disease’ above and ‘Role in more advanced disease’ above.)