PT  - JOURNAL ARTICLE
AU  - E.P. Judge
AU  - A. Fabre
AU  - H.I. Adamali
AU  - J.J. Egan
TI  - Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis
AID  - 10.1183/09031936.00115511
DP  - 2012 Jan 01
TA  - European Respiratory Journal
PG  - erj01155-2011
4099  - http://erj.ersjournals.com/content/early/2011/12/01/09031936.00115511.short
4100  - http://erj.ersjournals.com/content/early/2011/12/01/09031936.00115511.full
AB  - To evaluate the risk factors for and outcomes of acute exacerbations (AE) in patients with advanced idiopathic pulmonary fibrosis (IPF), and to examine the relationship between disease severity and neovascularization in explanted IPF lung tissue.IPF patients assessed for lung transplantation (n=55) were grouped into AE (n=27) and non-AE (NAE) groups. Haemodynamic data was collected at baseline, at time of AE and at lung transplantation. Histological analysis and CD31 immunostaining to quantify microvessel density (MVD) was performed on the explanted lung tissue of all transplanted patients (n=13).AE were associated with increased mortality (p=0.0015). Pulmonary hypertension (PH) at baseline and AE were associated with poor survival (p<0.01). PH at baseline was associated with a significant risk of AE (HR=2.217, p=0.041). Neovascularization was significantly increased in areas of cellular fibrosis and significantly decreased in honeycomb areas. There was a significant inverse correlation between mPAP and MVD in honeycomb areas.AE are associated with significantly increased mortality in patients with advanced IPF. PH is associated with the subsequent development of an AE and with poor survival. Both neovascularization and its correlation with mPAP vary significantly according to severity of fibrosis.