Authors
C Robalo Cordeiro, P Campos, L Carvalho, S Campainha, S Clemente, L Figueiredo, JM Jesus, A Marques, C Souto-Moura, R Pinto Basto, A Ribeiro, M Serrado, A Morais
Publication date
2016/3/1
Journal
Revista Portuguesa de Pneumologia (English Edition)
Volume
22
Issue
2
Pages
112-122
Publisher
Elsevier Doyma
Description
Idiopathic pulmonary fibrosis is a rare interstitial lung disease included in the Idiopathic Interstitial Pneumonias group. Although several potential risk factors have been described, it is a progressive fibrosing disease of unknown cause affecting mainly adults over 50 years and associated with a poor prognosis, reflected in a median survival of 2–3 years after diagnosis.
The concept of a multidisciplinary working group for the diagnosis of idiopathic pulmonary fibrosis is based on the need to have experienced pulmonologists, radiologists and pathologists in the evaluation and correct treatment of the disease, and requires the use of all available data about individual patients, standardized (largely through High Resolution Computed Tomography and pathology when needed) as well as non-standardized data (laboratory, serology and biomarkers). This approach helps to increase diagnostic accuracy and is an …
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