ICU Management of the Patient With Fibrotic Interstitial Lung Disease

Abstract

With the advent of new therapies and improvements in supportive care, survivorship in patients with interstitial lung disease (ILD) is increasing. However, this increase in number of patients living with ILD has resulted in an increase in the number of patients admitted to hospitals with acute exacerbations of ILD, most commonly manifested as advanced hypoxemic respiratory failure. In addition, patients with ILD may be admitted to the hospital as their first manifestation of ILD or progression of an ILD of yet to be diagnosed cause. All of these presentations carry significant risk of severe respiratory failure requiring admission to an ICU. It is therefore necessary for the critical care practitioner to have an approach to the patient with ILD being admitted to the ICU. This review summarizes an approach to the evaluation and management of patients presenting to the ICU through a discussion of: (1) diagnosis of acute exacerbation in patients with previously diagnosed ILD; (2) diagnosis of patients presenting with ILD of unknown cause; (3) treatment of both acute exacerbations and underlying causes of ILD; (4) supportive ICU care for advanced respiratory failure due to ILD; and (5) outcomes of patients with ILD and severe respiratory failure in the ICU. In addition, we offer suggested approaches to determining the cause of respiratory deterioration in patients with ILD and deciding which advanced respiratory support devices are reasonable in managing ILD patients who have progressive respiratory failure.