Outcome of Patients With Interstitial Lung Diseases Requiring Mechanical Ventilation

Abstract

In Brief Life-threatening episodes of acute respiratory failure may occur in patients with interstitial lung diseases, mainly idiopathic pulmonary fibrosis, and they may require admission to the intensive care unit and ventilatory support. Only a few studies evaluating the outcome of these patients have been published to date. This article provides a review of these data. The most frequent precipitating factor of acute respiratory failure is acute exacerbation of the disease (58%), followed by pulmonary infections (28%). Less frequently, respiratory failure is caused by pulmonary embolism, heart failure, pneumothorax, and anesthetic or diagnostic procedures. Eighty-five percent (77/91) of ventilated patients die in the intensive care unit, and the mortality rate of patients who are discharged is over 90%. The findings of these studies suggest that (1) management with noninvasive or invasive mechanical ventilation is highly unsuccessful in patients with pulmonary fibrosis and acute respiratory failure, and noninvasive ventilation avoids intubation in only a few patients; (2) a good outcome is only attained in cases with a reversible precipitating cause of respiratory insufficiency; (3) acute progression of fibrotic disease denotes a very poor outcome once mechanical ventilation has been implemented; and (4) in patients with pulmonary infections, early antibiotic treatment does not change the outcome. Without a clearly identified reversible cause of acute respiratory failure, initiating mechanical ventilation is of questionable value, except in patients in whom lung transplantation can be performed within a few days of the start of mechanical ventilation. Life-threatening episodes of acute respiratory failure may occur during the course of interstitial lung diseases, which may require admission to the intensive care unit and require ventilatory support. This article provides a review of the precipitating factors and therapeutic options in acute respiratory failure in these patients. The outcome of patients referred to the intensive care unit is extremely poor and not improved by mechanical ventilation. Without a clearly identified reversible cause of acute respiratory failure, initiating mechanical ventilation is of questionable value, except in patients in whom lung transplantation can be performed within a few days of the start of mechanical ventilation.