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Early diagnosis of idiopathic pulmonary fibrosis: Closer to the goal?

  • Alessia Comes
    Affiliations
    Unità Operativa Complessa di Pneumologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Largo Agostino Gemelli 8, 00168 Rome, Italy
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  • Giacomo Sgalla
    Affiliations
    Unità Operativa Complessa di Pneumologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Largo Agostino Gemelli 8, 00168 Rome, Italy
    Search for articles by this author
  • Luca Richeldi
    Correspondence
    Corresponding author.
    Affiliations
    Unità Operativa Complessa di Pneumologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Largo Agostino Gemelli 8, 00168 Rome, Italy
    Search for articles by this author
Published:August 23, 2020DOI:https://doi.org/10.1016/j.ejim.2020.08.018
      Early diagnosis of idiopathic pulmonary fibrosis (IPF) is definitely a challenge. Several studies have been investigating how to achieve this goal. IPF is the most common type of idiopathic interstitial pneumonia, the median age at diagnosis is approximately 65 years and it's more common in smokers and males [
      • Richeldi L.
      • Collard H.R.
      • Jones M.G.
      Idiopathic pulmonary fibrosis.
      ]. To date, there are only two therapeutic options available (pirfenidone and nintedanib) that have shown to be effectively slowing disease progression in terms of forced vital capacity (FVC) [
      • Raghu G.
      • Rochwerg B.
      • Zhang Y.
      • Garcia C.A.C.
      • Azuma A.
      • Behr J.
      • et al.
      An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline.
      ] with a further decrease in the risk of acute exacerbations for nintedanib [
      • Lamas D.J.
      • Kawut S.M.
      • Bagiella E.
      • Philip N.
      • Arcasoy S.M.
      • Lederer D.J.
      Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study.
      ]. Regardless of the disease severity, the delay between first respiratory symptoms and referral to a tertiary care centre could be years and this ‘waiting’ leads to an increase in mortality [
      • Richeldi L.
      • Du Bois R.M.
      • Raghu G.
      • Azuma A.
      • Brown K.K.
      • Costabel U.
      • et al.
      Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis.
      ]. Furthermore, the absence of accurate short-term indicators of therapeutic response leaves clinicians with uncertainty regarding management. Reduced survival time has been associated with various factors such as advanced age, severe physiological impairment, low body-mass index, radiological extent and severity of fibrosis determined by chest high-resolution computed tomography (HRCT), presence of comorbidities including pulmonary hypertension (PH), emphysema and bronchogenic cancer. Several risk prediction models have been developed in order to improve clinicians’ ability to predict prognosis in IPF and to modify the natural course of the disease, however accurate prognostication in patients with IPF is challenging due to the highly variable natural course of the disease [
      • Ley B.
      • Collard H.R.
      • King T.E.
      Clinical course and prediction of survival in idiopathic pulmonary fibrosis.
      ]. Formulating a diagnosis of IPF at an early stage and increasing prognostic accuracy are fundamental strategies to optimize clinical management and allow timely referral for lung transplantations.
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