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Editorials |

The Impact of Pulmonary Arterial Hypertension on Idiopathic Pulmonary Fibrosis

Charlene D. Fell, MD, MS, FCCP; Fernando J. Martinez, MD, MS, FCCP
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Affiliations: Calgary, AB, Canada ,  Ann Arbor, MI ,  Dr. Fell is Clinical Assistant Professor of Medicine, University of Calgary. Dr. Martinez is affiliated with the Division of Pulmonary and Critical Care Medicine, University of Michigan.

Correspondence to: Fernando J. Martinez, MD, MS, FCCP, University of Michigan, Pulmonary and Critical Care, TC 3916, 1500 E Medical Center Dr, Ann Arbor, MI 48109; e-mail: fmartine@umich.edu



Chest. 2007;131(3):641-643. doi:10.1378/chest.07-0057
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Idiopathic pulmonary fibrosis (IPF) is a progressive parenchymal lung disease of unknown etiology and poor prognosis.1 Poor prognosis has been associated with older age, male gender, lower FVC, lower diffusing capacity of the lung for carbon monoxide (Dlco), desaturation during exercise, and the extent of fibrosis seen on imaging studies.,15 The development of pulmonary arterial hypertension (PAH) in patients with pulmonary fibrosis is well recognized.3,6 Studies from the last few years78 have shown that PAH measured with right-heart catheterization or transthoracic echocardiography in IPF patients is associated with low Dlco, shorter walk distances, and desaturation during exercise. Similar relationships have been identified when brain natriuretic peptide was used as a surrogate marker for PAH in IPF patients.9 The optimal approach to identifying which IPF patients have PAH remains controversial, however. Nevertheless, increasing pulmonary artery pressure (PAP) is associated with an increasing risk of death in IPF patients,78 while elevated preoperative PAH may contribute to posttransplant mortality in IPF.10

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