Staging of acute exacerbation in patients with idiopathic pulmonary fibrosis

Lung. 2014 Feb;192(1):141-9. doi: 10.1007/s00408-013-9530-0. Epub 2013 Nov 13.


Background: The purpose of this study was to evaluate the predictors of a 3-month mortality rate of acute exacerbation of idiopathic pulmonary fibrosis (IPF) and provide a new staging system.

Methods: A total of 594 patients with IPF were included in this retrospective, observational study conducted from January 2001 to December 2010 at Okinawa Chubu Hospital.

Results: Among the 594 patients, 58 (9.8 %) developed acute exacerbation (AE) of IPF during the 10-year observation period. The median follow-up period for AE was 10.4 months. In-hospital mortality was 56.9 % and the 3-month mortality rate was 63.8 %. We identified the following four parameters in a multivariate analysis as: serum lactate dehydrogenase, sialylated carbohydrate antigen (KL-6), ratio of partial pressure of oxygen and fraction of inspiratory oxygen, and total extent of abnormal findings on high-resolution computed tomography of the chest. Patients were divided into two groups on the basis of the four composite parameters. Patients in the extensive disease-stage group required more mechanical ventilation and intensive therapy than those in the limited disease-stage group. The 3-month mortality rate was higher in patients in the extensive disease-stage group than in patients in the limited disease-stage group (80.6 vs. 54.5 %, respectively; p = 0.007).

Conclusions: Staging of AE in patients with IPF provided useful information regarding disease severity and short-term outcome.

Publication types

  • Observational Study

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Aged, 80 and over
  • Biomarkers / blood
  • Breath Tests
  • Chi-Square Distribution
  • Disease Progression
  • Female
  • Hospital Mortality
  • Humans
  • Idiopathic Pulmonary Fibrosis / blood
  • Idiopathic Pulmonary Fibrosis / diagnosis*
  • Idiopathic Pulmonary Fibrosis / mortality*
  • Idiopathic Pulmonary Fibrosis / therapy
  • Immunosuppressive Agents / therapeutic use
  • Japan
  • Kaplan-Meier Estimate
  • L-Lactate Dehydrogenase / blood
  • Length of Stay
  • Male
  • Middle Aged
  • Mucin-1 / blood
  • Multivariate Analysis
  • Oxygen / blood
  • Partial Pressure
  • Predictive Value of Tests
  • Prognosis
  • Proportional Hazards Models
  • Respiration, Artificial
  • Retrospective Studies
  • Risk Factors
  • Severity of Illness Index
  • Time Factors
  • Tomography, X-Ray Computed


  • Adrenal Cortex Hormones
  • Biomarkers
  • Immunosuppressive Agents
  • MUC1 protein, human
  • Mucin-1
  • L-Lactate Dehydrogenase
  • Oxygen