Acute Exacerbation of Interstitial Lung Disease: Early Diagnosis and Treatment

Diagnosis and treatment of acute exacerbation of interstitial lung disease (AE-ILD) continue to be challenging. The annual incidence of AE in idiopathic pulmonary fibrosis (IPF) is 5% to 15%, with an in-hospital mortality exceeding 50%. Similar annual incidence and mortality rates have been documented in other ILDs. The pathogenic mechanisms underlying AE are not entirely clear, although they could involve an acute injury or inflammatory process in previously affected lung tissue, with histological features of diffuse alveolar damage, similar to acute respiratory distress syndrome. AE-ILD is defined based on the following criteria: acute respiratory worsening within 30 days in a patient with a previous or concurrent diagnosis of ILD accompanied by new bilateral ground-glass abnormalities and/or consolidation on high-resolution computed tomography after ruling out heart failure or fluid overload. Pharmacologic treatments such as corticosteroids, antibiotics, and immunosuppressants have been and continue to be used despite scarce evidence from randomized placebo-controlled clinical trials. Oxygen therapy and ventilatory support are key elements of treatment of AE-ILD. The aim of our article is to provide an updated review on the diagnosis and treatment of AE-ILD and to propose practical algorithms for management.