BACKGROUND: The aim of this study was to assess the association between pulmonary vessel-related structures and cryptogenic fibrosing alveolitis (CFA) in a drug trial in a Chinese population using derived computed tomography (dCT) to evaluate functional reduction and survival.

MATERIAL AND METHODS: Discovery and validation cohorts were chosen separately by fulfilment of drug trial entry criteria, and we enrolled 269 and 292 consecutive patients, respectively. CFA patients who had undergone imaging based on volumetric non-contrast CT at our hospital were subjected to pulmonary vessel-related structure (PVS) measures and dCT to forecast mortality and reduction in reduced forced vital capacity of CFA.

RESULTS: The best forecaster of survival and reduction in terms of reduced forced vital capacity were found to be the dCT-generated outcomes in terms of PVS scores. Patients having less extensive disease highlighted the dCT outcomes through outperformance of CFA measures. When we used the cohort enhancement device, we found reduction in the requisite sample size of a CFA drug trial by 31% with the use of more than 5.0% dCT PVS score.

CONCLUSIONS: We found an association between CFA and PVS using dCT and it is far better than the results achieved so far by use of criterion standard measures. Additionally, reduction in the restrictive trial costs was also achieved by using cohort enhancement in a CFA drug trial setting, as PVS scores forced us to decrease the size of required CFA drug trial population by 30%. Interestingly, patients who had to take antifibrotic medication for longer periods had longer survival and less decreases forced vital capacity, as identified by PVS scores.

Keywords: idiopathic pulmonary fibrosis, multidetector computed tomography, Pulmonary Artery, Aged, 80 and over, Respiratory Function Tests, Tomography, X-Ray Computed, Vital Capacity