title: The earlier, the better: Impact of early diagnosis on clinical outcome in idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is a complex disease with a highly variable clinical course and generally poor prognosis. Classified as a rare disease, significant increases in incidence have been recorded worldwide in recent years. Left untreated IPF is extremely debilitating with substantial personal, social and economic implications.
  
  
  
  To discuss how IPF is diagnosed and managed in real life clinical practice with particular reference to Italy and to determine how new and effective therapies can be incorporated into a patient-centred management approach in order to improve the lives of patients with IPF.
  
  
  
  Barriers to early diagnosis are discussed. Cited reasons for delays in diagnosing IPF in Italy include: inherent difficulties in diagnosis; lack of knowledge/awareness of the condition among point-of-contact healthcare professionals; delays in referral to centres of excellence and underestimation of symptoms by both patients and healthcare workers. Valid therapeutic options with demonstrated efficacy in slowing the decline in lung function are now available for patients with IPF. The ASCEND trial confirmed the effects of pirfenidone, approved for the treatment of IPF on the basis of the four phase III trials. Nintedanib, a tyrosine kinase inhibitor that targets the PDGF receptors α/β, FGF receptors 1 to 3, and VEGF receptors 1-3, is approved in the USA and the EU for the treatment of IPF. The TOMORROW and the INPULSIS placebo controlled trials in patients with IPF confirm the efficacy and safety of nintedanib and recent interim analyses endorse its long-term effects in slowing disease progression.
  
  
  
  The importance of early and accurate diagnosis of IPF cannot be underestimated and it is the duty of all healthcare professionals to be vigilant to the symptoms of IPF and to involve a multidisciplinary team in diagnosing and managing IPF early in the course of disease.
authors.affiliation: Department of Clinical and Experimental Medicine, Respiratory Disease and Lung Function Unit, University of Parma, Italy. Electronic address: marina.aiello@unipr.it.
authors.forename: Marina
authors.initials: M
authors.lastname: Aiello
authors.affiliation: Department of Clinical and Experimental Medicine, Respiratory Disease and Lung Function Unit, University of Parma, Italy. Electronic address: giuseppina.bertorelli@unipr.it.
authors.forename: Giuseppina
authors.initials: G
authors.lastname: Bertorelli
authors.affiliation: Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University of Naples Federico II, Italy. Electronic address: Marialuisa.bocchino@unina.it.
authors.forename: Marialuisa
authors.initials: M
authors.lastname: Bocchino
authors.affiliation: Department of Clinical and Experimental Medicine, Respiratory Disease and Lung Function Unit, University of Parma, Italy. Electronic address: alfredoantonio.chetta@unipr.it.
authors.forename: Alfredo
authors.initials: A
authors.lastname: Chetta
authors.affiliation: Direttore UOC di Pneumologia ed UTSIR, ASL 01 Abruzzo OC San Salvatore, L'Aquila, Italy. Electronic address: FioreDonati@yahoo.it.
authors.forename: Alfeo
authors.initials: A
authors.lastname: Fiore-Donati
authors.affiliation: Department of Clinical and Experimental Medicine- Lung Disease Unit, University of Sassari, Italy. Electronic address: Fois.Ale@libero.it.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Fois
authors.affiliation: Pneumology Department, SS Annunziata Hospital, University of Chieti, Italy. Electronic address: StefanoMarinari@alice.it.
authors.forename: Stefano
authors.initials: S
authors.lastname: Marinari
authors.affiliation: Cardiothoracic and Vascular Department, Pneumology Unit, IRCCS Policlinico San Matteo Foundation, Pavia, Italy. Electronic address: oggionni@smatteo.pv.it.
authors.forename: Tiberio
authors.initials: T
authors.lastname: Oggionni
authors.affiliation: Department of Pneumology, AO "SS. Antonio e Biagio", Alessandria, Italy. Electronic address: bpolla@ospedale.al.it.
authors.forename: Biagio
authors.initials: B
authors.lastname: Polla
authors.affiliation: Department of Cardiology and Thoracic Medicine, Respiratory Disease Unit, AOU Careggi, Florence, Italy. Electronic address: rosiel@aou-careggi.toscana.it.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Rosi
authors.affiliation: Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University of Naples Federico II, Italy. Electronic address: stanziola@unina.it.
authors.forename: Anna
authors.initials: A
authors.lastname: Stanziola
authors.affiliation: Cardio-Thoracic Department, Fondazione Policlinico Universitario "A. Gemelli", Roma, Italy. Electronic address: FrancescoVarone@hotmail.com.
authors.forename: Francesco
authors.initials: F
authors.lastname: Varone
authors.affiliation: Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University of Naples Federico II, Italy. Electronic address: sanduzzi@unina.it.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Sanduzzi
mesh_terms: D018450:Disease Progression; D042241:Early Diagnosis; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D007211:Indoles / Q000627:therapeutic use*; D007558:Italy; D010348:Patient Care Team; D018802:Patient-Centered Care; D011379:Prognosis; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011728:Pyridones / Q000627:therapeutic use*
chemical_list: D007211:Indoles; D047428:Protein Kinase Inhibitors; D011728:Pyridones; C093844:pirfenidone; C530716:nintedanib
keywords: Diagnosis; INPULSIS; Idiopathic pulmonary fibrosis; Nintedanib; Pirfenidone; TOMORROW