Marina Aiello1, Giuseppina Bertorelli2, Marialuisa Bocchino3, Alfredo Chetta4, Alfeo Fiore-Donati5, Alessandro Fois6, Stefano Marinari7, Tiberio Oggionni8, Biagio Polla9, Elisabetta Rosi10, Anna Stanziola11, Francesco Varone12, Alessandro Sanduzzi13. 1. Department of Clinical and Experimental Medicine, Respiratory Disease and Lung Function Unit, University of Parma, Italy. Electronic address: marina.aiello@unipr.it. 2. Department of Clinical and Experimental Medicine, Respiratory Disease and Lung Function Unit, University of Parma, Italy. Electronic address: giuseppina.bertorelli@unipr.it. 3. Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University of Naples Federico II, Italy. Electronic address: Marialuisa.bocchino@unina.it. 4. Department of Clinical and Experimental Medicine, Respiratory Disease and Lung Function Unit, University of Parma, Italy. Electronic address: alfredoantonio.chetta@unipr.it. 5. Direttore UOC di Pneumologia ed UTSIR, ASL 01 Abruzzo OC San Salvatore, L'Aquila, Italy. Electronic address: FioreDonati@yahoo.it. 6. Department of Clinical and Experimental Medicine- Lung Disease Unit, University of Sassari, Italy. Electronic address: Fois.Ale@libero.it. 7. Pneumology Department, SS Annunziata Hospital, University of Chieti, Italy. Electronic address: StefanoMarinari@alice.it. 8. Cardiothoracic and Vascular Department, Pneumology Unit, IRCCS Policlinico San Matteo Foundation, Pavia, Italy. Electronic address: oggionni@smatteo.pv.it. 9. Department of Pneumology, AO "SS. Antonio e Biagio", Alessandria, Italy. Electronic address: bpolla@ospedale.al.it. 10. Department of Cardiology and Thoracic Medicine, Respiratory Disease Unit, AOU Careggi, Florence, Italy. Electronic address: rosiel@aou-careggi.toscana.it. 11. Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University of Naples Federico II, Italy. Electronic address: stanziola@unina.it. 12. Cardio-Thoracic Department, Fondazione Policlinico Universitario "A. Gemelli", Roma, Italy. Electronic address: FrancescoVarone@hotmail.com. 13. Department of Clinical Medicine and Surgery, Section of Respiratory Disease, University of Naples Federico II, Italy. Electronic address: sanduzzi@unina.it.
Abstract
BACKGROUND: 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. OBJECTIVES: 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. OUTCOMES: 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. CONCLUSIONS: 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.
BACKGROUND: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. OBJECTIVES: 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. OUTCOMES: 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. CONCLUSIONS: 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: Rossella Di Bidino; Paola Rogliani; Alfredo Sebastiani; Alberto Ricci; Francesco Varone; Giacomo Sgalla; Bruno Iovene; Teresa Bruni; Maria Chiara Flore; Michela D'Ascanio; Francesco Cavalli; Daniela Savi; Loreta Di Michele; Americo Cicchetti; Luca Richeldi Journal: Front Med (Lausanne) Date: 2022-06-09
Authors: Catharina C Moor; Marlies S Wijsenbeek; Elisabetta Balestro; Davide Biondini; Benjamin Bondue; Vincent Cottin; Ron Flewett; Liam Galvin; Steve Jones; Maria Molina-Molina; Lurdes Planas-Cerezales; Antje Prasse; Helmut Prosch; Anne-Marie Russell; Michel Viegas; Guenther Wanke; Wim Wuyts; Michael Kreuter; Francesco Bonella Journal: ERJ Open Res Date: 2019-10-21
Authors: Charlotte A Easton-Jones; John E Madigan; Samantha Barnum; Lara K Maxwell; Sandra D Taylor; Terry Arnesen; Nicola Pusterla Journal: J Vet Intern Med Date: 2018-09-17 Impact factor: 3.333