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Recent Advances in Chest Medicine |

Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis*

Nina M. Patel, MD; David J. Lederer, MD, MS; Alain C. Borczuk, MD; Steven M. Kawut, MD, MS, FCCP
Author and Funding Information

*From the Departments of Medicine (Dr. Patel) and Pathology (Dr. Borczuk), College of Physicians and Surgeons, and the Department of Medicine (Drs. Lederer and Kawut), College of Physicians and Surgeons, Joseph L. Mailman School of Public Health, Columbia University, New York, NY.

Correspondence to: Nina M. Patel, MD, Division of Pulmonary, Allergy & Critical Care, Columbia University, 622 W 168th St, PH 8 East, Room 101, New York, NY 10032; e-mail: np2199@columbia.edu



Chest. 2007;132(3):998-1006. doi:10.1378/chest.06-3087
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Idiopathic pulmonary fibrosis (IPF) is an untreatable diffuse parenchymal lung disease with a median survival of < 3 years. Pulmonary hypertension (PH) is frequently seen in patients with IPF and is commonly attributed to hypoxic vasoconstriction and capillary destruction. Pathology findings include endothelial proliferation and medial hypertrophy that exceed those expected in the setting of hypoxia. Noninvasive evaluation has limited sensitivity and specificity for the diagnosis of PH in IPF; therefore, right-heart catheterization remains the “gold standard” diagnostic test. PH in patients with IPF is associated with decreased exercise capacity and worse survival. Given the grave consequences of this condition, treatment of PH could improve functional outcomes and survival. However, possible treatments such as long-term supplemental oxygen and targeted vascular therapy are either unstudied or remain unproven.

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