Interstitial Lung Disease: A Focused Review for the Emergency Clinician

Interstitial lung disease (ILD) is a group of restrictive pulmonary diseases associated with diffuse interstitial and parenchymal inflammation. Patients can present to the emergency department with severe exacerbation.

This narrative review provides emergency clinicians with the most recent evidence concerning acute exacerbation of ILD (AE-ILD).

AE-ILD can present as acute respiratory distress in a patient with a pre-existing ILD diagnosis or as a de novo presentation of ILD, and is associated with significant morbidity and mortality. A variety of underlying triggers may result in AE-ILD. Emergency clinicians must first assess for extraneous causes of respiratory decompensation prior to diagnosing AE-ILD. For a de novo presentation of ILD, emergency physicians should also assess for possible reversible causes. AE-ILD is managed with systemic steroids, immunosuppressants, intravenous antibiotics, supplemental oxygen, and extracorporeal membrane oxygenation in severe cases. Given the high mortality rates in the absence of lung transplantation, early referral to transplant centers is essential to increase chances of survival.

Emergency clinician knowledge of AE-ILD can improve the evaluation and management of these patients.