title: [Novel therapies for idiopathic pulmonary fibrosis].
abstract: Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease of unknown cause. The clinical course is unpredictable, but the disease is usually progressive with a median survival of 2-5 years as a result of advanced respiratory failure. The current hypothesis of the disease mechanism is recurrent injury to the respiratory epithelium which leads to an uncontrolled wound healing process resulting in fibrosis rather than repair. Despite better understanding of the pathogenesis, there is no effective therapy for the disease. In this review, we present the positive results of recently published clinical trials regarding therapy for IPF, with emphasis on pirfenidone and nintedanib.
authors.forename: Ori
authors.initials: O
authors.lastname: Wand
authors.forename: Mordechai R
authors.initials: MR
authors.lastname: Kremer
mesh_terms: D018450:Disease Progression; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000503:physiopathology; D007211:Indoles / Q000627:therapeutic use*; D011728:Pyridones / Q000627:therapeutic use*; D020545:Respiratory Mucosa / Q000473:pathology; D015996:Survival Rate
chemical_list: D007211:Indoles; D011728:Pyridones; C093844:pirfenidone; C530716:nintedanib