Significance of Combined Emphysema in Idiopathic Pulmonary Fibrosis and Serum Surfactant Protein-D as a Prognostic Factor.
Idiopathic pulmonary fibrosis is a chronic disease of unknown aetiology and is often associated with a syndrome called combined pulmonary fibrosis and emphysema. This study aimed to identify practical
predictors of prognosis in IPF patients associated with CPFE.
We retrospectively studied 72 patients with IPF and evaluated the threshold of emphysematous area affecting prognosis on high-resolution computed tomography scans. As predictor candidates, various pulmonary function tests and biomarkers, e.g. surfactant protein-A and SP-D, were assessed.
The survival rate of the CPFE group, defined as having an emphysematous area greater than 25% on HRCT, was significantly worse than that of the non-CPFE group, despite no significant difference in fibrosis scores. An annual percent decline of diffusing capacity of the lung for carbon monoxide of more than 5% was a significant prognostic factor in the CPFE group.
High concentration of serum SP-D was a significant prognostic factor in both the CPFE and non-CPFE groups. However, cut-off levels in the CPFE group were lower than those in the non-CPFE group.
We demonstrated worse prognosis in IPF associated with CPFE syndrome
compared to the other subset of IPF, and showed that % DLCO and SP-D are useful predictors of poor prognosis.
Pulm Res Respir Med Open J. 2017; 4(2): 24-32. doi: 10.17140/PRRMOJ-4-135