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

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.

Ventilatory support and mechanical properties of the fibrotic lung acting as a “squishy ball”

“…Interestingly, in the patients with fibrotic lung and superimposed DAD, retrospective data showed an association between higher PEEP levels and mortality [16]. Compared to ARDS, physiology of MV in IPF patients is much less known [3], and it is unclear whether opening and closing of alveolar units during tidal breathing occurs, as what exactly the role of PEEP is on alveolar recruitment.…”

“…Overall, AE-ILD has a poor prognosis and the choice to initiate MV or to admit the patient to the ICU can be challenging, particularly in patients with IPF [3]. In several clinical settings, ICU physicians tend to be reluctant to admit IPF patients if they are not already listed for transplant, considering invasive ventilation as a bridge-totransplant therapy [16].…”

“…Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of idiopathic ILD, often worsened by acute exacerbation episodes (AE-IPF). During these dramatic events, the typical usual interstitial pneumonia pattern (UIP)-the radiologic and histologic hallmark of IPF is overlapped with diffuse alveolar damage (DAD), sharing similarities with the acute respiratory distress syndrome (ARDS) [3]. Little is known about the outcome of latter patients receiving MV, and the influence of the extent of lung fibrosis component on ventilator management [4].…”

E-selectin as a prognostic factor of patients hospitalized due to acute inflammatory respiratory diseases: a single institutional study

“…The prognosis of interstitial pneumonia is extremely poor. The mortality ratio of acute exacerbation of idiopathic pulmonary fibrosis (IPF), the most significant disease among interstitial pneumonia, is 80 % (Enomoto et al, 2018[6]; Atsumi et al, 2018[1]; Marchioni et al, 2018[11]). Moreover, 40 % of patients with IPF die from acute exacerbation (Natsuizaka et al, 2014[13]).…”

Patients with interstitial lung disease and pulmonary sarcoidosis are at high risk for severe illness related to COVID-19

“…11 Giving antibiotics and steroids in acute exacerbations of ILD has been common practice for many years, but steroid treatment has come under scrutiny as of late, with several trials showing possible harm and increased risk of serious adverse reactions. 12,13 Histologically, most acute exacerbations of ILD are characterized by diffuse alveolar damage, while others are characterized by organizing pneumonia, alveolar hemorrhage, and unspecifi c infl ammatory changes. Clinical judgment should be used, and steroids could be considered in cases more likely to respond, such as ILD related to exacerbation of underlying connective tissue disease, which may involve more organizing pneumonia or alveolar hemorrhage.…”