Abstract

Interstitial lung diseases (ILDs) are a heterogeneous group of more than 200 diseases characterized by widespread fibrotic and inflammatory abnormalities of the lung. In this post COVID era, the number of patients with ILD is increasing possibly due to improved diagnosis, management and survival as well as due to the enhanced awareness of lung health. All of these patients carried significant risk of exacerbations and hospitalization. Especially, those with advanced ILD may develop acute hypoxemic respiratory failure that may warrant admission to the ICU with significant morbidity and mortality.

The diagnosis and management of ILD in the ICU present unique challenges. Careful clinical evaluation is pivotal for diagnosis, identifying potential triggers and for differential diagnoses. Though the diagnosis primarily relies on chest CT imaging, bronchoalveolar lavage and transbronchial or surgical lung biopsy may also be informative but pose significant procedural risks.

A significant gap remains in our understanding of the management of ILD, particularly in the ICU setting and requires a multidisciplinary approach. Corticosteroids are the cornerstone of treatment for acute exacerbations of most ILDs. Additional antifibrotics and immunosuppressive agents may be used in cases with compelling indications. Supplemental oxygen, particularly with high-flow nasal cannula, is often employed to manage severe hypoxemia. Noninvasive ventilation can be useful for worsening hypoxemia and/or hypercapnia. When mechanical ventilation is used, it is recommended to target low tidal volumes to minimize lung injury and associated with high mortality rate. Prone positioning, careful fluid management, infection control and addressing concomitant pulmonary hypertension are essential components of care. Extracorporeal membrane oxygenation is a high-risk intervention reserved for the most severe cases usually as a bridge to Lung transplantation, the last realistic hope for long-term survival.