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. 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. Key Points•CT scan imaging of the chest is the first-line test in the diagnosis of an acute exacerbation of idiopathic pulmonary fibrosis.•The diagnostic approach to ICU patients without a previous diagnosis of interstitial lung disease should proceed with caution, specifically for the performance of bronchoscopy or open lung biopsy during critical illness given the risk of worsening respiratory failure with these procedures.•Rapidly progressive interstitial lung disease should be considered in the differential diagnosis of critically ill adults with ARDS.•High-flow nasal cannula use is associated with fewer discontinuations and increased oral intake and ability to communicate compared with noninvasive ventilation in critically ill adults with interstitial lung disease.•Short- and long-term mortality is high (30-day mortality, 78%; 6-month mortality, 96%) in patients with interstitial lung disease receiving invasive mechanical ventilation. •CT scan imaging of the chest is the first-line test in the diagnosis of an acute exacerbation of idiopathic pulmonary fibrosis.•The diagnostic approach to ICU patients without a previous diagnosis of interstitial lung disease should proceed with caution, specifically for the performance of bronchoscopy or open lung biopsy during critical illness given the risk of worsening respiratory failure with these procedures.•Rapidly progressive interstitial lung disease should be considered in the differential diagnosis of critically ill adults with ARDS.•High-flow nasal cannula use is associated with fewer discontinuations and increased oral intake and ability to communicate compared with noninvasive ventilation in critically ill adults with interstitial lung disease.•Short- and long-term mortality is high (30-day mortality, 78%; 6-month mortality, 96%) in patients with interstitial lung disease receiving invasive mechanical ventilation. Interstitial lung disease (ILD) is a broad group of diffuse parenchymal lung injury patterns characterized by varying degrees of inflammation and fibrosis. ILDs with a fibrotic phenotype are characterized by features on chest CT scan imaging such as reticulation, traction bronchiectasis, and honeycombing.1Solomon J.J. Fischer A. Connective tissue disease-associated interstitial lung disease: a focused review.J Intensive Care Med. 2015; 30: 392-400Crossref PubMed Google Scholar A fibrotic phenotype can be found in several distinct ILDs, including idiopathic pulmonary fibrosis (IPF), idiopathic interstitial pneumonias, connective tissue disease-associated ILD (CTD-ILD), fibrotic hypersensitivity pneumonitis (fHP), pneumoconioses, medication- or drug-induced ILDs, sarcoidosis, postinfectious ILD, radiation-induced ILD, smoking-related ILD, and unclassifiable ILDs.2Spagnolo P. Distler O. Ryerson C.J. et al.Mechanisms of progressive fibrosis in connective tissue disease (CTD)-associated interstitial lung diseases (ILDs).Ann Rheum Dis. 2021; 80: 143-150Crossref PubMed Scopus (108) Google Scholar,3Raghu G. Remy-Jardin M. Richeldi L. et al.Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT Clinical Practice Guideline.Am J Respir Crit Care Med. 2022; 205: e18-e47Crossref PubMed Scopus (526) Google ScholarCLINICAL QUESTIONA 59-year-old man with idiopathic pulmonary fibrosis taking nintedanib and 2 L/min of oxygen is admitted to the ICU for acute chronic hypoxemic respiratory failure. Three days prior to presentation, the patient developed increased dyspnea, fevers, and myalgias after spending time with his grandchild who had been experiencing symptoms of a respiratory tract infection. Currently, he is afebrile, normotensive, and has an oxygen saturation of 92% on high-flow nasal cannula (50 L of flow, Fio2 0.7).Which of the following is the most appropriate diagnostic test?A: Bronchoscopy with transbronchial lung biopsyB: CT scan of the chestC: Thoracic ultrasoundD: Open lung biopsyE: Serum C-reactive protein A 59-year-old man with idiopathic pulmonary fibrosis taking nintedanib and 2 L/min of oxygen is admitted to the ICU for acute chronic hypoxemic respiratory failure. Three days prior to presentation, the patient developed increased dyspnea, fevers, and myalgias after spending time with his grandchild who had been experiencing symptoms of a respiratory tract infection. Currently, he is afebrile, normotensive, and has an oxygen saturation of 92% on high-flow nasal cannula (50 L of flow, Fio2 0.7). Which of the following is the most appropriate diagnostic test? A: Bronchoscopy with transbronchial lung biopsyB: CT scan of the chestC: Thoracic ultrasoundD: Open lung biopsyE: Serum C-reactive protein Acute exacerbation of ILD (AE-ILD) may occur at any time during the of in acute hypoxemic respiratory failure may admission to the ICU with significant and et in patients with acute exacerbation of interstitial lung disease admitted to the 2021; PubMed Scopus Google Scholar This is in and is acute exacerbation of can occur in of fibrotic ILDs as A. Acute exacerbation of interstitial lung disease in the care J Crit Care Med. 2022; PubMed Google Scholar is for the to are an acute worsening of the days characterized by new without an Ryerson C.J. et exacerbation of idiopathic pulmonary fibrosis. J Respir Crit Care Med. PubMed Scopus Google et exacerbation of interstitial lung disease after Med. PubMed Scopus Google G. Acute exacerbation in interstitial lung PubMed Scopus Google Scholar of lung may diffuse on the underlying fibrotic A. 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