Pirfenidone treatment of idiopathic pulmonary fibrosis

Pirfenidone is the first antifibrotic agent to be approved for the treatment of pulmonary fibrosis. Idiopathic pulmonary fibrosis (IPF) is one of the idiopathic interstitial pneumonias with the worst prognoses; approximately half of patients die within 3-5 years, and the need for an effective treatment has been unmet until recently. The etiology of IPF is still unknown and its pathogenesis is poorly understood. Anti-inflammatory drugs, such as corticosteroids and some immunosuppressants, have been empirically used to treat IPF, although they have not been objectively proven to be effective by large-scale randomized, controlled trials. Pirfenidone is an agent that can inhibit the decline of forced vital capacity (FVC)/vital capacity (VC) and that thereby can be hoped to decrease the mortality rate. The number of clinical trials of pirfenidone completed, ongoing, or planned is growing, and the present status of pirfenidone as treatment for IPF is summarized in this review.