title: Idiopathic Pulmonary Fibrosis: Treatment and Prognosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with a prognosis that can be worse than for many cancers. The initial stages of the condition were thought to mainly involve chronic inflammation; therefore, corticosteroids and other drugs that have anti-inflammatory and immunosuppressive actions were used. However, recently, agents targeting persistent fibrosis resulting from aberrant repair of alveolar epithelial injury have been in the spotlight. There has also been an increase in the number of available antifibrotic treatment options, starting with pirfenidone and nintedanib. These drugs prevent deterioration but do not improve IPF. Therefore, nonpharmacologic approaches such as long-term oxygen therapy, pulmonary rehabilitation, and lung transplantation must be considered as additional treatment modalities.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mie University Graduate School of Medicine, Edobashi Tsu, Mie, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Fujimoto
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mie University Graduate School of Medicine, Edobashi Tsu, Mie, Japan.
authors.forename: Tetsu
authors.initials: T
authors.lastname: Kobayashi
authors.affiliation: Department of Pulmonary Medicine, Infectious Disease and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.
authors.forename: Arata
authors.initials: A
authors.lastname: Azuma
keywords: GAP score; clinical trial; idiopathic pulmonary fibrosis; treatment