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Open Access 01-12-2018 | Review

Acute exacerbation of idiopathic pulmonary fibrosis: lessons learned from acute respiratory distress syndrome?

Authors: Alessandro Marchioni, Roberto Tonelli, Lorenzo Ball, Riccardo Fantini, Ivana Castaniere, Stefania Cerri, Fabrizio Luppi, Mario Malerba, Paolo Pelosi, Enrico Clini

Published in: Critical Care | Issue 1/2018

Abstract

Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease characterized by progressive loss of lung function and poor prognosis. The so-called acute exacerbation of IPF (AE-IPF) may lead to severe hypoxemia requiring mechanical ventilation in the intensive care unit (ICU). AE-IPF shares several pathophysiological features with acute respiratory distress syndrome (ARDS), a very severe condition commonly treated in this setting.

A review of the literature has been conducted to underline similarities and differences in the management of patients with AE-IPF and ARDS.

During AE-IPF, diffuse alveolar damage and massive loss of aeration occurs, similar to what is observed in patients with ARDS. Differently from ARDS, no studies have yet concluded on the optimal ventilatory strategy and management in AE-IPF patients admitted to the ICU. Notwithstanding, a protective ventilation strategy with low tidal volume and low driving pressure could be recommended similarly to ARDS. The beneficial effect of high levels of positive end-expiratory pressure and prone positioning has still to be elucidated in AE-IPF patients, as well as the precise role of other types of respiratory assistance (e.g., extracorporeal membrane oxygenation) or innovative therapies (e.g., polymyxin-B direct hemoperfusion). The use of systemic drugs such as steroids or immunosuppressive agents in AE-IPF is controversial and potentially associated with an increased risk of serious adverse reactions.

Common pathophysiological abnormalities and similar clinical needs suggest translating to AE-IPF the lessons learned from the management of ARDS patients. Studies focused on specific therapeutic strategies during AE-IPF are warranted.

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Title: Acute exacerbation of idiopathic pulmonary fibrosis: lessons learned from acute respiratory distress syndrome?
Authors: Alessandro Marchioni
Roberto Tonelli
Lorenzo Ball
Riccardo Fantini
Ivana Castaniere
Stefania Cerri
Fabrizio Luppi
Mario Malerba
Paolo Pelosi
Enrico Clini
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: Critical Care / Issue 1/2018
Electronic ISSN: 1364-8535
DOI: https://doi.org/10.1186/s13054-018-2002-4

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Acute exacerbation of idiopathic pulmonary fibrosis: lessons learned from acute respiratory distress syndrome?

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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