Introduction

Few studies describe whether the extent of radiologic fibrosis correlates with other measures of disease severity in patients with idiopathic pulmonary fibrosis (IPF). A direct correlation could confirm that the clinical and physiological limitations in these patients are attributed to the extent of fibrosis, rather than co-morbidities, such as emphysema, or subjective factors.

Objectives

To assess the correlation between a visual fibrosis score and lung function, quality of life and symptoms.

Methods

Patients with IPF were recruited from the Danish PFBIO cohort. The HRCT scans were scored by two experienced thoracic radiologists using a standardized form and blinded to patient data, each-others scores and the time of scan. The extents of reticulation, traction bronchiectasis and honeycombing were combined in a composite quantitative fibrosis score. Dyspnoea was assessed in a questionnaire on a scale from 0 to 5.

Results

In the 66 included patients, fibrosis score was correlated with FVC (r=-0.35, p=0.005), DLCO (r=-0.53, p<0.001), St. George?s Respiratory Questionnaire (r=0.46, p=0.002) and dyspnoea (r=0.51, p<0.001). There was no significant difference between patients with or without concomitant emphysema.

Conclusions

A visual fibrosis score in IPF correlates with lung function tests, quality of life and dyspnoea, further validating these measurements as directly related to the extent of lung fibrosis.