Acute Hypoxemic Respiratory Failure: Idiopathic Pulmonary Fibrosis

  • Battistina Farris


Idiopathic pulmonary fibrosis (IPF) is a disease characterized by widespread fibrotic and inflammatory abnormalities of the lung. A multidisciplinary approach is particularly important when deciding to perform additional diagnosis assessments, integrating biopsy results with clinical and CT feature. Respiratory failure is a common complication in advanced stages: oxygen supplementation and ventilator support need to be titrated according to the severity of ARF. High-Flow Nasal oxygen may be an alternative to conventional oxygen therapy in patients requiring high-flow and high oxygen concentration to correct hypoxemia and control dyspnea. Neither Non-Invasive Ventilation (NIV) nor Invasive Mechanical Ventilation (IMV) seem to change the poor outcomes associate to advanced stages of IPF, however, in selected patients, such as those with less severe ARF a NIV trial might help in the patients who may present a better short-term prognosis. More invasive techniques, including IMV and Extracorporeal Membrane Oxygenation (ECMO) should be limited to patients listed for lung transplant.


  1. 1.
    Kaunisto J, Salomaa E-R, Hodgson U, Kaarteenaho R, Mylarniemi M. Idiopathic pulmonary fibrosis—a systematic review on methodology the collection of epidemiological data. BMC Pulm Med. 2013;13:53.CrossRefGoogle Scholar
  2. 2.
    Garcia CK. Idiopathic pulmonary fibrosis: update on genetic discoveries. Proc Am Thorac Soc. 2011;8:158–62.CrossRefGoogle Scholar
  3. 3.
    Walsh SL, Wells AU, Desai SR, et al. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study. Lancet Respir Med. 2016;4:557–65.CrossRefGoogle Scholar
  4. 4.
    Zappala CJ, Latsi PI, Nicholson AG, et al. Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis. Eur Respir J. 2010;35:830–6.CrossRefGoogle Scholar
  5. 5.
    Raghu G, Collard HP, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788–824.CrossRefGoogle Scholar
  6. 6.
    Raghu G, Remi-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guidelines. Am J Respir Crit Care Med. 2018;198:e44–68.CrossRefGoogle Scholar
  7. 7.
    Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6(2):138–53.CrossRefGoogle Scholar
  8. 8.
    Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidences, risk factors and outcome. Eur Respir J. 2011;37:356–63.CrossRefGoogle Scholar
  9. 9.
    Lederer D, Martinez FJ. Idiopathic pulmonary fibrosis. N Engl J Med. 2018;378:1811–23.CrossRefGoogle Scholar
  10. 10.
    Richeldi L, du Bois LM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2071–82.CrossRefGoogle Scholar
  11. 11.
    Frat JP, Coudrov R, Marianovic N, Thille AW. High-flow nasal oxygen therapy and noninvasive ventilation in the management of acute hypoxemic respiratory failure. Ann Transl Med. 2017;5(14):297.CrossRefGoogle Scholar
  12. 12.
    Vianello A, Arcano G, Battistella L, Vio S, Concas A, et al. Noninvasive ventilation in the event of acute respiratory failure in patients with idiopathic pulmonary fibrosis. J Crit Care. 2014;29:562–7.CrossRefGoogle Scholar
  13. 13.
    Faverio P, De Giacomi F, Sardella L, Fiorentini G, Carone M, et al. Management of acute respiratory failure in interstitial lung diseases: overview and clinical insights. BMC Pulm Med. 2018;18:70.CrossRefGoogle Scholar
  14. 14.
    Vianello A, Arcano G, Paladini L, Iovino S. Successful management of acute respiratory failure in an idiopathic pulmonary fibrosis patient using an extracorporeal carbon dioxide removal system. Sarcoidosis Vasc Diffuse Lung Dis. 2016;33:186–90.PubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Battistina Farris
    • 1
  1. 1.Department of Respiratory MedicineSta. Barbara Hospital IglesiasIglesiasItaly

Personalised recommendations