Acute exacerbation of Idiopathic Pulmonary Fibrosis: lessons learned from the Acute Respiratory Distress Syndrome

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 the Acute Respiratory Distress Syndrome (ARDS), a very severe condition commonly treated in this setting. A review of the literature has been here conducted to underline similarities and differences for caring and managing patients with AE-IPF and ARDS. During AE-IPF, a diffuse alveolar damage and massive loss of aeration occurs, similarly to what is observed in patients with ARDS. Differently from ARDS, no studies have concluded yet 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 these patients, as well as the precise role of other types of respiratory assistance (e.g. ECMO) or innovative therapies (e.g. polymyxin-B direct hemoperfusion). The use of systemic drugs such as steroids or immunosuppressive agents in AE-IPF is controverial and potentially associated with increased risk of serious adverse reactions. Common pathophysiological abnormalities and similar clinical needs suggest translating to AE-IPF the lessons learned from ARDS patients. Studies focused on specific therapeutic strategies during AE-IPF are warranted.