The earlier, the better: Impact of early diagnosis on clinical outcome in idiopathic pulmonary fibrosis

Marina Aiello; Giuseppina Bertorelli; Marialuisa Bocchino; Alfredo Chetta; Alfeo Fiore-Donati; Alessandro Fois; Stefano Marinari; Tiberio Oggionni; Biagio Polla; Elisabetta Rosi; Anna Stanziola; Francesco Varone; Alessandro Sanduzzi

doi:10.1016/j.pupt.2017.02.005

The earlier, the better: Impact of early diagnosis on clinical outcome in idiopathic pulmonary fibrosis

Pulm Pharmacol Ther. 2017 Jun:44:7-15. doi: 10.1016/j.pupt.2017.02.005. Epub 2017 Feb 28.

Authors

Affiliations

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.

PMID: 28257817
DOI: 10.1016/j.pupt.2017.02.005

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.

Keywords: Diagnosis; INPULSIS; Idiopathic pulmonary fibrosis; Nintedanib; Pirfenidone; TOMORROW.

Publication types

Review

MeSH terms

Disease Progression
Early Diagnosis
Humans
Idiopathic Pulmonary Fibrosis / diagnosis*
Idiopathic Pulmonary Fibrosis / drug therapy
Idiopathic Pulmonary Fibrosis / physiopathology
Indoles / therapeutic use*
Italy
Patient Care Team
Patient-Centered Care
Prognosis
Protein Kinase Inhibitors / therapeutic use
Pyridones / therapeutic use*

Substances

Indoles
Protein Kinase Inhibitors
Pyridones
pirfenidone
nintedanib