title: Therapeutic Approach to Adult Fibrotic Lung Diseases.
abstract: Among the interstitial lung diseases (ILDs), idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis, and fibrotic connective tissue disease-related ILD are associated with a worse prognosis, with death occurring as a result of both respiratory failure and serious associated comorbidities. The recent development and approval of the antifibrotic agents nintedanib and pirfenidone, both of which reduced the rate of decline in lung function in patients with IPF in clinical trials, offer hope that it may be possible to alter the increased mortality associated with IPF. Although chronic hypersensitivity pneumonitis and connective tissue disease related-ILD may be associated with an inflammatory component, the evidence for the use of immunosuppressive agents in their treatment is largely limited to retrospective studies. The lack of benefit of immunosuppressive therapy in advanced fibrosis argues for rigorous clinical trials using antifibrotic therapies in these types of ILD as well. Patients with fibrotic ILD may benefit from identification and management of associated comorbid conditions such as pulmonary hypertension, gastroesophageal reflux, and OSA, which may improve the quality of life and, in some cases, survival in affected individuals. Because early assessment may optimize posttransplantation outcomes, lung transplant evaluation should occur early in patients with IPF and those with other forms of fibrotic ILD.
authors: Section of Pulmonary and Critical Care, Department of Medicine, University of Chicago, Chicago, IL. Electronic address: aadegunsoye@medicine.bsd.uchicago.edu.
authors: Ayodeji
authors: A
authors: Adegunsoye
authors: Section of Pulmonary and Critical Care, Department of Medicine, University of Chicago, Chicago, IL.
authors: Mary E
authors: ME
authors: Strek
mesh_terms: D000542:Alveolitis, Extrinsic Allergic / Q000188:drug therapy; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D003240:Connective Tissue Diseases / Q000188:drug therapy; D018450:Disease Progression; D004791:Enzyme Inhibitors / Q000627:therapeutic use*; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007211:Indoles / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000188:drug therapy*; D011379:Prognosis; D011728:Pyridones / Q000627:therapeutic use*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D004791:Enzyme Inhibitors; D007166:Immunosuppressive Agents; D007211:Indoles; D011728:Pyridones; C093844:pirfenidone; C530716:nintedanib
keywords: connective tissue disease; hypersensitivity pneumonia; idiopathic interstitial pneumonia; idiopathic pulmonary fibrosis; interstitial lung disease