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    Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis.

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    Authors
    Judge, Eoin P
    Fabre, Aurelie
    Adamali, Huzaifa I
    Egan, Jim J
    Affiliation
    Dept of Respiratory Medicine, and Irish National Lung Transplant Unit, Mater Misericordiae University Hospital, Eccles Street, Dublin 7, Ireland. eoinjudge@yahoo.com
    Issue Date
    2012-07
    
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    Citation
    Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis. 2012, 40 (1):93-100 Eur. Respir. J.
    Journal
    The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology
    URI
    http://hdl.handle.net/10147/252612
    DOI
    10.1183/09031936.00115511
    PubMed ID
    22135282
    Abstract
    The aim of this study was to evaluate the risk factors for and outcomes of acute exacerbations in patients with advanced idiopathic pulmonary fibrosis (IPF), and to examine the relationship between disease severity and neovascularisation in explanted IPF lung tissue. 55 IPF patients assessed for lung transplantation were divided into acute (n=27) and non-acute exacerbation (n=28) groups. Haemodynamic data was collected at baseline, at the time of acute exacerbation and at lung transplantation. Histological analysis and CD31 immunostaining to quantify microvessel density (MVD) was performed on the explanted lung tissue of 13 transplanted patients. Acute exacerbations were associated with increased mortality (p=0.0015). Pulmonary hypertension (PH) at baseline and acute exacerbations were associated with poor survival (p<0.01). PH at baseline was associated with a significant risk of acute exacerbations (HR 2.217, p=0.041). Neovascularisation (MVD) was significantly increased in areas of cellular fibrosis and significantly decreased in areas of honeycombing. There was a significant inverse correlation between mean pulmonary artery pressure and MVD in areas of honeycombing. Acute exacerbations were associated with significantly increased mortality in patients with advanced IPF. PH was associated with the subsequent development of an acute exacerbation and with poor survival. Neovascularisation was significantly decreased in areas of honeycombing, and was significantly inversely correlated with mean pulmonary arterial pressure in areas of honeycombing.
    Item Type
    Article
    Language
    en
    ISSN
    1399-3003
    ae974a485f413a2113503eed53cd6c53
    10.1183/09031936.00115511
    Scopus Count
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    Mater Misericordiae Hospital

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