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First published online on February 22, 2007
Chest, doi:10.1378/chest.06-1466
A more recent version of this article appeared on March 1, 2007
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Significance of Pulmonary Arterial Pressure and Diffusion Capacity of the Lung as Prognosticator in Patients with Idiopathic Pulmonary Fibrosis

Kunio Hamada, MD, PhD1; Sonoko Nagai, MD, PhD2; Shigeru Tanaka, MD3; Tomohiro Handa, MD, PhD2; Michio Shigematsu, MD, PhD4; Taishi Nagao, MD, PhD5; Michiaki Mishima, MD, PhD2; Masanori Kitaichi, MD, PhD6 and Takateru Izumi, MD, PhD7

1Chitose City Hospital, Division of Internal Medicine, Chitose, Hokkaido, Japan 2Kyoto University Hospital, Department of Respiratory Medicine, Kyoto, Japan 3Moju-kai Tanaka Clinic, Osaka, Japan 4Sumitomo Hospital, Division of Respiratory Medicine, Osaka, Japan 5Shiga University of Medical Science, Department of Respiratory Medicine, Otsu, Shiga, Japan 6Kinki Chest Disease Center, Sakai, Osaka, Japan 7Central Clinic in Kyoto, Kyoto, Japan

kunio.hamada{at}city.chitose.hokkaido.jp

Abstract

Study objectivesTo evaluate the long-term clinical courses of patients with idiopathic pulmonary fibrosis (IPF) complicated with pulmonary arterial hypertension (PAH).

DesignProspective analysis of consecutive IPF patients undergoing initial workups containing right heart catheterization (RHC) and pulmonary function tests (PFT). Pulmonary arterial pressure (PAP) and diffusion capacity of the lung for carbon monoxide (DLCO) were focused on.

SettingUniversity hospital.

PatientsSeventy-eight patients with IPF (67 men, 11 women; 59 pathologically diagnosed, 19 clinically) had been followed after initial workups for a maximum of 14 years.

Measurements and resultsRHC data on 61 patients and PFT data on 52 patients were available. Five-year survival rates were 62.2% in normal-PAP group (mean PAP below 17 mmHg, n=37) and 16.7% in high-PAP group (mean PAP above 17 mmHg, n=24) (p<0.001), 70.4% in preserved-DLCO group (percentage predicted above 40, n=27) and 20.0% in low-DLCO group (percentage predicted below 40, n=25) (p<0.001), and 82.6% in Group 1 (normal PAP and preserved DLCO, n=23) and 15.6% in Group 2 (high PAP, low DLCO, or both, n=32) (p<0.0001). The relative risk of mortality within 5 years after RHC was 2.20 (95% CI 1.40-3.45) in the high-PAP group, 2.70 (95% CI 1.46-4.99) in the low-DLCO group, and 4.85 (95% CI 1.97-11.97) in Group 2.

ConclusionDLCO was a critical factor for evaluating disease status and prognosis and PAP status provided feasible information in initial workups of IPF patients.

Key Words: idiopathic pulmonary fibrosis • pulmonary arterial hypertension • prognosis


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