Diagnostic criteria for idiopathic pulmonary fibrosis ipf. Idiopathic pulmonary fibrosis is a devastating, agerelated lung disease of unknown cause that has few treatment options. We searched pubmed from january, 1996, to april, 2011, using the search terms. Idiopathic pulmonary fibrosis ipf is defined as a specific form of chronic. Idiopathic pulmonary fibrosis ipf is a rare, chronic, progressive, interstitial lung disease of unknown etiology affecting around five million patients worldwide, affecting more men than women. Key updates on guidelines and therapeutics find, read and cite all the research you need on researchgate. Idiopathic pulmonary fibrosis european respiratory society. This disease was once thought to be a chronic inflammatory process, but current evidence indicates that the fibrotic response is driven by abnormally activated alveolar epithelial cells aecs.
These cells produce mediators that induce the formation of fibroblast and. Idiopathic means there is no known cause at this time. Pulmonary fibrosis can be caused by an identifiable irritation to the lungs, but in many cases the cause is unknown. Idiopathic pulmonary fibrosis ipf is defined as a specific form of chronic, progressive fibrosing.
Idiopathic pulmonary fibrosis ipf is a steadily progressive and ultimately fatal disease of unknown origin. Idiopathic pulmonary fibrosis may be a disease of recurrent. Ipf, idiopathic pulmonary fibrosis, ild, interstitial lung disease. Executive summary ganesh raghu, martine remyjardin, jeffrey l. An official atsersjrsalat clinical practice guideline.
Key updates on guidelines and therapeutics concluding remarks article pdf available in respiratory research 141 april 20 with 14 reads. Idiopathic pulmonary fibrosis ipf is a chronic, progressive disease. Idiopathic pulmonary fibrosis american thoracic society. In cases when the cause of pf is unknown, the diagnosis is idiopathic pulmonary fibrosis ipf. Diagnosis of idiopathic pulmonary fibrosis american thoracic.
Idiopathic pulmonary fibrosis ipf represents a field of constant. Scarring in alveoli prevents oxygen from passing into blood vessel. Idiopathic pulmonary fibrosis ipf is an interstitial lung disease characterised by chronic, progressive scarring of the lungs and the pathological. Pdf an official atsersjrsalat clinical practice guideline. Pdf on apr 16, 20, u costabel and others published idiopathic pulmonary fibrosis in 2011.