Idiopathic pulmonary fibrosis is a progressive disease with a median survival of 2-3 years. There is no universally accepted tool for identifying individuals with a poorer prognosis. du Bois et al (1) proposed a 4 point scoring system to predict 1 year mortality. This tool requires external validation.
Data was collected on 94 consecutive patients with IPF. The du Bois score was calculated based on age, respiratory hospitalisation, baseline forced vital capacity (FVC) and 6 month change in FVC. The predictive value of the score was assessed using the area under the receiver operator characteristic curve (AUC). Patients were followed up for at least 3 years or until death.
Median age was 69 years (interquartile range 64-76). 57.4% were male. 1 year mortality was 10.6%. The 1 year mortality was 0% for those patients scoring 0-14 points, 11.5% for those with 16-21 points, 23% for those scoring 22-29 and 21% for those scoring>30 points.
For prediction of 1 year mortality, the AUC was 0.76 (95% CI 0.67-0.85,p=0.004) indicating moderate predictive value. For prediction of 3 year mortality, AUC was 0.72 (0.64-0.78,p=0.0005).

This study validates a novel risk score for prediction of outcome in patients with idiopathic pulmonary fibrosis.
1) du Bois, R.M. et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;184:459-466.