title: Pharmacologic therapies for idiopathic pulmonary fibrosis, past and future.
abstract: Idiopathic pulmonary fibrosis (IPF) is a severe, progressive fibrotic disease of the lung of unknown etiology that affects approximately 150,000 patients in the United States. It carries a median survival of two to three years, but clinical course can vary markedly from patient to patient. There has been no established treatment for IPF, but recent advances in coordinated clinical trials through groups such as IPFnet and academia-industry partnerships have allowed this relatively rare disease to be studied in much greater depth. Historically, the default therapy for IPF was a combination of prednisone, N-acetylcysteine, and azathioprine, but recent trials have shown that this regimen actually increases mortality. An enormous body of work in recent years, spanning the bench to the bedside, has radically altered our understanding of the molecular mechanisms underlying IPF. Newer modalities, particularly those involving monoclonal antibodies targeted at specific pathways known to contribute to the fibrotic process, have generated a great deal of excitement in the field, and recent clinical trials on therapies such as pirfenidone and nintedanib herald a new era in targeted IPF therapies.
authors.affiliation: Department of Medicine, Division of Pulmonary, Allergy & Critical Care Medicine, Emory University School of Medicine , Atlanta, GA 30322 , USA.
authors.forename: Bashar S
authors.initials: BS
authors.lastname: Staitieh
authors.forename: Elisabetta A
authors.initials: EA
authors.lastname: Renzoni
authors.forename: Srihari
authors.initials: S
authors.lastname: Veeraraghavan
mesh_terms: D000818:Animals; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D058990:Molecular Targeted Therapy; D016032:Randomized Controlled Trials as Topic
chemical_list: D000893:Anti-Inflammatory Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D000998:Antiviral Agents; D004791:Enzyme Inhibitors; D007166:Immunosuppressive Agents
keywords: Idiopathic pulmonary fibrosis; N-acetylcysteine; nintedanib; pirfenidone; prednisone