Abstract
CPFE is a syndrome with distinct clinical, functional and radiological characteristics. Recent works have demonstrated the impact of pulmonary hypertension (PH) on worse survival but little is known about the morphological and molecular substrates. The aim of our study was to evaluate the arterial remodelling and expression of transforming growth factor-beta1 (TGF-β) in the lungs of patients with CPFE compared to IPF without PH. The study was performed on lung samples or native lungs (2007-10 recruitment period): 7 CPFE patients (mean age 59±5.8 yrs), 15 IPF patients (mean age 53±10yrs) and 5 non-implanted donors (mean age 30±10yrs) as a normal control group. In all cases morphometry was used to measure arterial intimal, medial, and total thickening and immunohistochemical evaluation of TGF-β was quantified in macrophages and alveolar epithelial cells. In CPFE, FEV1%pred and FVC%pred were significantly less reduced than in IPF (p=0.004 and p=0.003, respectively), while DLCO%pred was similar. Intimal and total thickening were significantly increased in CPFE than IPF (p=0.004 and p=0.03, respectively). A trend of increased alveolar TGF-β expression was detected in CPFE compared to IPF (p=0.07) while its expression in macrophages was similar. As expected, arterial remodelling and TGF-βexpression were significantly increased in both IPF (p=0.001; p=0.001 respectively) and CPFE (p=0.004; p=0.003, respectively) compared to controls. In conclusion, our study demonstrated that lungs with CPFE display significant arterial remodelling which may represent pathological substrate for the increased occurrence of PH.
- © 2011 ERS