Acute exacerbation of idiopathic pulmonary fibrosis: high-resolution CT scores predict mortality

Eur Radiol. 2012 Jan;22(1):83-92. doi: 10.1007/s00330-011-2211-6. Epub 2011 Aug 7.


Objectives: To determine high-resolution computed tomography (HRCT) findings helpful in predicting mortality in patients with acute exacerbation of idiopathic pulmonary fibrosis (AEx-IPF).

Methods: Sixty patients with diagnosis of AEx-IPF were reviewed retrospectively. Two groups (two observers each) independently evaluated pattern, distribution, and extent of HRCT findings at presentation and calculated an HRCT score at AEx based on normal attenuation areas and extent of abnormalities, such as areas of ground-glass attenuation and/or consolidation with or without traction bronchiectasis or bronchiolectasis and areas of honeycombing. The correlation between the clinical data including the HRCT score and mortality (cause-specific survival) was evaluated using the univariate and multivariate Cox-regression analyses.

Results: Serum KL-6 level, PaCO(2), and the HRCT score were statistically significant predictors on univariate analysis. Multivariate analysis revealed that the HRCT score was an independently significant predictor of outcome (hazard ratio, 1.13; 95% confidence interval, 1.06-1.19, P = 0.0002). The area under receiver operating characteristics curve for the HRCT score was statistically significant in the classification of survivors or nonsurvivors (0.944; P < 0.0001). Survival in patients with HRCT score ≥245 was worse than those with lower score (log-rank test, P < 0.0001).

Conclusion: The HRCT score at AEx is independently related to prognosis in patients with AEx-IPF.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Disease
  • Adult
  • Aged
  • Aged, 80 and over
  • Analysis of Variance
  • Blood Gas Analysis / methods
  • Bronchiectasis / blood
  • Bronchiectasis / diagnostic imaging*
  • Bronchiectasis / mortality
  • C-Reactive Protein / metabolism
  • Disease Progression
  • Female
  • Humans
  • Idiopathic Pulmonary Fibrosis / diagnostic imaging*
  • Idiopathic Pulmonary Fibrosis / metabolism
  • Idiopathic Pulmonary Fibrosis / mortality
  • L-Lactate Dehydrogenase / blood
  • Male
  • Middle Aged
  • Mucin-1 / blood
  • Odds Ratio
  • Predictive Value of Tests
  • Prognosis
  • Proportional Hazards Models
  • ROC Curve
  • Respiratory Insufficiency / blood
  • Respiratory Insufficiency / diagnostic imaging*
  • Respiratory Insufficiency / metabolism
  • Respiratory Insufficiency / mortality*
  • Retrospective Studies
  • Survival Analysis
  • Tomography, X-Ray Computed*


  • MUC1 protein, human
  • Mucin-1
  • C-Reactive Protein
  • L-Lactate Dehydrogenase