Acute Respiratory Failure in Interstitial Lung Disease Complicated by Pulmonary Hypertension

Interstitial lung disease represents a group of diffuse parenchymal lung diseases with overwhelming morbidity and mortality when complicated by acute respiratory failure. Recently, trials investigating outcomes and their determinants have provided insight into these high mortality rates. Pulmonary hypertension is a known complication of interstitial lung disease and there is high prevalence in idiopathic pulmonary fibrosis, connective tissue disease, and sarcoidosis subtypes. Interstitial lung disease associated pulmonary hypertension has further increased mortality with acute respiratory failure, and there is limited evidence to guide management. This review describes investigations and management of interstitial lung disease associated acute respiratory failure complicated by pulmonary hypertension. Despite the emerging attention on interstitial lung disease associated acute respiratory failure and the influence of pulmonary hypertension, critical care management remains a clinical and ethical challenge.