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Therapeutic Update in Idiopathic Pulmonary Fibrosis.
MedLine Citation:
PMID:  21222174     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Idiopathic pulmonary fibrosis (IPF) is a disease of the elderly with a mean age at presentation of 66 years. It is the most common type of idiopathic lung fibrosis, and the most lethal, with a median survival of 3 to 5 years after diagnosis. Abnormalities in fibroblast and humoral response mechanisms may play a role in the pathogenesis of fibrosis in IPF. Clinical trials suggest that pirfenidone, an oral antifibrotic agent, N-acetylcysteine, an antioxidant and perhaps anticoagulation, may have some beneficial effect; however, large-scale studies are necessary for confirmation. Immunosuppression with corticosteroids likely does not confer benefit. Lung transplantation has been shown to improve survival in selected IPF patients. Comorbidities accompanying IPF include gastroesophageal reflux, sleep disturbance, pulmonary arterial hypertension, and coronary artery disease amongst others, and ought to be promptly recognized and managed appropriately. While the US Food and Drug Administration has not currently approved any treatments for IPF, patients with IPF should continue to be strongly encouraged to enroll in ongoing clinical trials for this devastating disease.
Authors:
Andrew L Chan; Rokhsara Rafii; Samuel Louie; Timothy E Albertson
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-1-11
Journal Detail:
Title:  Clinical reviews in allergy & immunology     Volume:  -     ISSN:  1559-0267     ISO Abbreviation:  -     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-1-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9504368     Medline TA:  Clin Rev Allergy Immunol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Division of Pulmonary, Critical Care and Sleep Medicine, UC Davis School of Medicine, 4150 V Street, Suite 3400, Sacramento, CA, USA, andrew.chan@ucdmc.ucdavis.edu.
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