Management of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing lung disorder characterized by progressive dyspnea, exercise intolerance and, ultimately, respiratory failure and death. The incidence of IPF seems to be increasing, whereas its etiology remains unelucidated. Agents capable of modulating...

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Bibliographic Details
Published inThe American journal of the medical sciences Vol. 341; no. 6; p. 450
Main Authors Fioret, Daniel, Perez, Rafael L, Roman, Jesse
Format Journal Article
LanguageEnglish
Published United States 01.06.2011
Subjects
Antitussive Agents - therapeutic use
Disease Progression
Humans
Idiopathic Pulmonary Fibrosis - drug therapy
Idiopathic Pulmonary Fibrosis - epidemiology
Idiopathic Pulmonary Fibrosis - surgery
Idiopathic Pulmonary Fibrosis - therapy
Lung Transplantation
Oxygen - administration & dosage
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Summary:Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing lung disorder characterized by progressive dyspnea, exercise intolerance and, ultimately, respiratory failure and death. The incidence of IPF seems to be increasing, whereas its etiology remains unelucidated. Agents capable of modulating inflammation, kinase pathways, vascular tone, coagulation and fibrosis have been tested in clinical studies although not always in large, randomized, placebo-controlled prospective trials. Despite this effort, a therapy capable of improving survival remains elusive. Consequently, the management of IPF focuses on the early identification of subjects for lung transplantation and on the treatment of comorbidities such as hypoxemia, cough and deconditioning. Until effective therapies are identified, patients and referring physicians are urged to consider participation in well-designed clinical trials.
ISSN:1538-2990
DOI:10.1097/MAJ.0b013e31821fbdbc