Abstract
Interstitial lung disease (ILD) encompasses a diverse group of parenchymal lung diseases characterized by varying degrees of inflammation and/or fibrosis. Patients with ILD frequently require hospitalization, with many needing intensive care unit (ICU) admission, most often due to respiratory failure. The diagnosis and management of ILD in the ICU present unique challenges. Diagnosis primarily relies on chest CT imaging to identify fibrosis and inflammation. Acute exacerbations, whether in idiopathic pulmonary fibrosis (IPF) or non-IPF ILD, require careful evaluation of potential triggers and differential diagnoses. Bronchoalveolar lavage may provide valuable information, such as the identification of infections, but carries risks of complications. Biopsies, whether transbronchial or surgical, can also be informative but pose significant procedural risks. Corticosteroids are the cornerstone of treatment for acute exacerbations of IPF, with higher doses potentially benefiting non-IPF ILD. Additional immunosuppressive agents may be used in cases with evidence of inflammation. Oxygen supplementation, particularly with high-flow nasal cannula, is often employed to manage severe hypoxemia, while 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; high PEEP may be less effective and even associated with increased mortality. Prone positioning can improve oxygenation in severely hypoxemic patients. In addition to ventilatory strategies, careful fluid management and addressing concomitant pulmonary hypertension are essential components of care. Extracorporeal membrane oxygenation is a high-risk intervention reserved for the most severe cases. Lung transplantation may be considered for end-stage ILD patients in the ICU, with outcomes dependent on the urgency of transplantation and the patient’s overall condition. Managing ILD in the ICU requires a multidisciplinary approach, and despite recent advances, mortality remains high, emphasizing the need for continued research and individualized treatment strategies.
Key Points
- ILD Overview: Encompasses diverse diseases categorized into fibrosis- and inflammation-predominant types, with idiopathic pulmonary fibrosis (IPF) being a key example of the former.
- Acute Exacerbations: Acute worsening of ILD, particularly in IPF, is a common ICU admission cause, with triggers including infections, toxins, or idiopathic factors.
- Diagnostic Tools: High-resolution CT is pivotal for identifying exacerbations and differentiating fibrosis from inflammation. Bronchoalveolar lavage and biopsies provide additional diagnostic value but carry risks.
- Corticosteroid Use: Steroids are the cornerstone for managing exacerbations, especially in IPF, despite limited evidence from randomized trials. High-dose regimens are common but require caution in infection-prone patients.
- Ventilatory Strategies: Low tidal volume ventilation minimizes barotrauma. High PEEP may be detrimental due to non-recruitable alveolar units, emphasizing individualized approaches.
- Advanced Interventions: Extracorporeal membrane oxygenation (ECMO) serves as a bridge to lung transplantation in severe cases but carries significant risks.
- Antifibrotic and Antibiotic Use: Antifibrotics reduce long-term disease progression but are less effective during acute exacerbations. Empirical antibiotics target infections like Pneumocystis jirovecii.
- Pulmonary Hypertension Management: Inhaled vasodilators like nitric oxide improve ventilation-perfusion matching, while high PEEP and intravenous vasodilators are generally avoided.
- Lung Transplantation: Offers a salvage option for end-stage ILD, with outcomes influenced by patient selection, timing, and perioperative management.
- Prognosis and Research Needs: Mortality remains high, especially in IPF patients requiring mechanical ventilation. Future research should focus on biomarkers, advanced therapies, and personalized ICU strategies.
