Combined Pulmonary Fibrosis and
Enphysema (CPFE): correlation
between TC parameters and
functional decline
Gaetano Rea, Maria D'Amato, Maurizia Lanza, Anna agnese Stanziola, Antonio Molino European Respiratory Journal 2017 50: PA3731; DOI: 10.1183/1393003.congress-2017.PA3731
Abstract
Introduction:The Combined Pulmonary Fibrosis and Enphysema (CPFE)is a typical example of “coexisting pattern” of great importance from a clinical point of view.
Aim: The goal of our work is to evaluate patients with CPFE and any difference between the subgroup with the lower lobes not UIP pattern and the subgroup with UIP pattern. In addition, the clinical course of patients with CPFE and the two subgroups was correlated with TCHR quantitative parameters. Materials and Methods: 33 patients with satisfied pathophysiological and radiological criteria of CPFE were recruited. All patients were submitted to HRCT at the time of diagnosis and to follow up visits every 6 months for a period of 3 years, collecting lung function and cardiology data. The CT scan was performed with 64-MDCT with volumetric acquisition, thickness 1.25 mm and technical “state of the art” for the study of diffuse infiltrative changes, with kernel and reconstruction filters dedicated. HRTC validated radiological indices as the MLD (mean lung density), the skewness and kurtosis were evaluated. These data were analyzed by a radiologist with expertise of ILDs disease and analyzed by the software Image J. Then compared with lung function and cardiology data.
Results: The analysis of quantitative and qualitative parameters confirmed different CPFE clinical entities with a greater severity in CPFE group with pattern UIP to the lower lobes.
Conclusion: This data could have a role in deciding the best policy options and treatment including the ability to access new target antifibrotic drugs for this patients that show a clinical and prognostic behavior similar to patients with IPF.
