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Severe idiopathic pulmonary fibrosis: A clinical approach

  • R. Lipsi
    Affiliations
    U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe-MultiMedica IRCCS, Via San Vittore, 12, 20123 Milan, Italy
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  • D. Mazzola
    Affiliations
    U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe-MultiMedica IRCCS, Via San Vittore, 12, 20123 Milan, Italy
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  • A. Caminati
    Affiliations
    U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe-MultiMedica IRCCS, Via San Vittore, 12, 20123 Milan, Italy
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  • D. Elia
    Affiliations
    U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe-MultiMedica IRCCS, Via San Vittore, 12, 20123 Milan, Italy
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  • C. Lonati
    Affiliations
    U.O. di Medicina Generale, Ospedale San Giuseppe-MultiMedica IRCCS, Via San Vittore, 12, 20123 Milan, Italy
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  • S. Harari
    Correspondence
    Corresponding author at: U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe-MultiMedica IRCCS, Via San Vittore, 12, 20123 Milan, Italy.
    Affiliations
    U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe-MultiMedica IRCCS, Via San Vittore, 12, 20123 Milan, Italy

    U.O. di Medicina Generale, Ospedale San Giuseppe-MultiMedica IRCCS, Via San Vittore, 12, 20123 Milan, Italy
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Published:January 18, 2018DOI:https://doi.org/10.1016/j.ejim.2018.01.014

      Abstract

      Idiopathic pulmonary fibrosis (IPF) is a devastating progressive disease associated with a high mortality rate. Novel antifibrotic therapies have been recently demonstrated to slow disease progression and improve survival. However, the management of IPF remains a difficult challenge, since lung complications can still occur, particularly in patients with advanced-stage disease. This paper highlights the most common complications and difficult tasks related to severe IPF such as acute exacerbation of the disease, development of lung cancer, rapid disease progression, and indication for lung transplantation.

      Abbreviations:

      IPF (idiopathic pulmonary fibrosis), UIP (Usual Interstitial Pneumonia), FVC (Forced Vital Capacity), SVC (Slow Vital Capacity), FEV1 (Forced Expiratory Volume in the first second), TLC (Total Lung Capacity), RV (Residual Volume), DLCO (Diffusing Capacity of the Lung for Carbon Monoxide), 6-MWT (6-Minute Walk Test), EF (Ejection Fraction), TRV (Tricuspid Regurgitation Peak Velocity), TAPSE (Tricuspid Annular Plane Systolic Excursion), ABG (Arterial Blood Gas analysis), O2 (Oxygen therapy), pCO2 (Carbon Dioxide Partial Pressure), pO2 (Oxygen Partial Pressure), SpO2 (Oxygen Saturation), RA (Room Air), CBC (Complete Blood Count), ERS (Erythrocyte Sedimentation Rate), CRP (C-Reactive Protein), ANA (AntiNuclear Antibodies), ENA (Extractable Nuclear Antigen antibodies), RF (Rheumatoid Factor), BNP (Brain Natriuretic Peptide), AE (Acute Exacerbation), ARDS (Acute Respiratory Distress Syndrome), HRCT (High Resolution Computed Tomography), PFT (Pulmonary Function Test), L (Liter), BSA (Body Surface Area), PCR (Polymerase Chain Reaction)

      Keywords

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