Prognosis of Patients with Idiopathic Pulmonary Fibrosis Admitted to the Intensive Care Unit

Abstract

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive diffuse parenchymal lung disease of unknown etiology. Most IPF patients develop a progressive restrictive lung disease as a result of progressive lung fibrosis, and respiratory failure eventually mandates mechanical ventilation. This study was aimed at studying the clinical features of patients with IPF admitted to the intensive care unit (ICU) due to acute respiratory failure, so as to investigate their mortality and prognostic factors. Materials and Methods: We retrospectively reviewed the medical records of all patients with IPF admitted to the medical CU of National Taiwan University Hospital, Taipei, Taiwan, from January 1999 to April 2003. Results: A total of 13 eligible patients, including 10 men, with a median age of 72(56-87) years, were identified. Nine patients had been on corticosteroids already at the time of admission. The most common precipitating factor for respiratory failure was pneumonia. Sepsis developed in 11(85%) patients, while septic shock developed in 6(46%), and multiple organ failure occurred in 7(54%). Seven patients (54%) received immunosuppressive therapy in the ICU. The ICU and hospital mortality rates were 62% and 85%, respectively. Univariate analysis showed that the development of septic shock and the increased number of cases of organ failure were associated with significantly higher mortality. Multivariate survival analysis showed that the development of septic shock (hazard ratio=16.69; p=0.04) and the increasing number of organ failure (HR=3.42 per additional dysfunctional organ system; p=0.04) were independent factors associated with significantly higher mortality. Conclusions: These findings suggest that IPF patients admitted to the ICU with acute respiratory failure have a poor prognosis in the presence of septic shock or multiple organ failure.