title: Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis: Joint Consensus of Sociedade Portuguesa de Pneumologia, Sociedade Portuguesa de Radiologia e Medicina Nuclear e Sociedade Portuguesa de Anatomia Patológica.
abstract: 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 internationally accepted recommendation. In regard to therapy, the situation has changed radically since the publication of the ATS/ERS/JRS/ALAT 2011 guidelines on the diagnosis and management of idiopathic pulmonary fibrosis where it was stressed that no proven therapy exists for this disease. Currently besides non-pharmacological treatment, therapy of complications and comorbidities and palliative care, nintedanib and pirfenidone, two compounds with pleiotropic mechanisms of action, are to date, the two drugs with confirmed efficacy in slowing functional decline and disease progression in idiopathic pulmonary fibrosis patients.
authors.affiliation: Centre of Pulmonology, Coimbra University Hospital, Coimbra, Portugal. Electronic address: carlos.crobalo@gmail.com.
authors.forename: C
authors.initials: C
authors.lastname: Robalo Cordeiro
authors.affiliation: Imagiology Department, Santa Marta Hospital, Northern Lisbon Hospital Centre, Lisbon, Portugal.
authors.forename: P
authors.initials: P
authors.lastname: Campos
authors.affiliation: Anatomical Pathology Institute, Faculty of Medicine of the University of Coimbra, Coimbra, Portugal.
authors.forename: L
authors.initials: L
authors.lastname: Carvalho
authors.affiliation: Pulmonology Department, Vila Nova de Gaia/Espinho Hospital Centre, Vila Nova de Gaia, Portugal.
authors.forename: S
authors.initials: S
authors.lastname: Campainha
authors.affiliation: Pulmonology Department, Beatriz Ângelo Hospital, Loures, Portugal.
authors.forename: S
authors.initials: S
authors.lastname: Clemente
authors.affiliation: Radiology Department, Santa Marta Hospital, Central Lisbon Hospital Centre, Lisbon, Portugal.
authors.forename: L
authors.initials: L
authors.lastname: Figueiredo
authors.affiliation: Radiology Department, São João Hospital Centre, Oporto, Portugal.
authors.forename: J M
authors.initials: JM
authors.lastname: Jesus
authors.affiliation: Pulmonology Department, São João Hospital Centre, Faculty of Medicine of Porto University, Oporto, Portugal.
authors.forename: A
authors.initials: A
authors.lastname: Marques
authors.affiliation: Pathology Department, São João Hospital Centre, Faculty of Medicine of Porto University, Oporto, Portugal.
authors.forename: C
authors.initials: C
authors.lastname: Souto-Moura
authors.affiliation: Pulmonology II Department, Pulido Valente Hospital, Northern Lisbon Hospital Centre, Lisbon, Portugal.
authors.forename: R
authors.initials: R
authors.lastname: Pinto Basto
authors.affiliation: Anatomical Pathology Department, Pulido Valente Hospital, Northern Lisbon Hospital Centre, Lisbon, Portugal.
authors.forename: A
authors.initials: A
authors.lastname: Ribeiro
authors.affiliation: Pulmonology II Department, Pulido Valente Hospital, Northern Lisbon Hospital Centre, Lisbon, Portugal.
authors.forename: M
authors.initials: M
authors.lastname: Serrado
authors.affiliation: Pulmonology Department, São João Hospital Centre, Faculty of Medicine of Porto University, Oporto, Portugal.
authors.forename: A
authors.initials: A
authors.lastname: Morais
mesh_terms: D000465:Algorithms; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000628:therapy*
keywords: Diagnosis; Guideline; Idiopathic pulmonary fibrosis; Therapeutics