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* From Chitose City Hospital, Division of Internal Medicine (Dr. Hamada), Chitose, Hokkaido; Kyoto University Hospital, Department of Respiratory Medicine (Drs. Nagai, Handa, and Mishima), Kyoto; Moju-kai Tanaka Clinic (Dr. Tanaka), Osaka; Sumitomo Hospital, Division of Respiratory Medicine (Dr. Shigematsu), Osaka; Shiga University of Medical Science, Department of Respiratory Medicine (Dr. Nagao), Otsu, Shiga; Kinki Chest Disease Center (Dr. Kitaichi), Sakai, Osaka; and Central Clinic in Kyoto (Dr. Izumi), Kyoto, Japan.
Correspondence to: Kunio Hamada, MD, PhD, Division of Internal Medicine, Chitose City Hospital, Hokko 2–1-1, Chitose, Hokkaido, 066-0033, Japan; e-mail kunio.hamada{at}city.chitose.hokkaido.jp
Abstract
Study objectives: To evaluate the long-term clinical course of patients with idiopathic pulmonary fibrosis (IPF) complicated with pulmonary arterial hypertension.
Design: Prospective analysis of consecutive IPF patients undergoing initial workup with right-heart catheterization (RHC) and pulmonary function testing (PFT). Pulmonary arterial pressure (PAP) and diffusion capacity of the lung for carbon monoxide (DLCO) were focused on.
Setting: University hospital.
Patients: Seventy-eight patients with IPF (67 men, 11 women; diagnosis by pathology, n = 59; clinical diagnosis, n = 19) had been followed up after initial workup for a maximum of 14 years.
Measurements and results: RHC data on 61 patients and PFT data on 52 patients were available. Five-year survival rates were 62.2% in the normal-PAP group (mean PAP < 17 mm Hg, n = 37) and 16.7% in the high-PAP group (mean PAP > 17 mm Hg, n = 24) [p < 0.001]; 70.4% in the preserved-DLCO group (percentage of predicted > 40%, n = 27) and 20.0% in the low-DLCO group (percentage of predicted < 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 risks of mortality within 5 years after RHC were 2.20 (95% confidence interval [CI], 1.40 to 3.45) in the high-PAP group, 2.70 (95% CI, 1.46 to 4.99) in the low-DLCO group, and 4.85 (95% CI, 1.97 to 11.97) in group 2.
Conclusion: DLCO was a critical factor for evaluating disease status and prognosis, and PAP status provided feasible information in the initial workup of IPF patients.
Key Words: idiopathic pulmonary fibrosis • prognosis • pulmonary arterial hypertension
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