| Budget Amount *help |
¥3,250,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥150,000)
Fiscal Year 2007: ¥650,000 (Direct Cost: ¥500,000、Indirect Cost: ¥150,000)
Fiscal Year 2006: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 2005: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 2004: ¥1,600,000 (Direct Cost: ¥1,600,000)
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| Research Abstract |
Background: Recently, increasing numbers of clinical investigations of combined pulmonary fibrosis and emphysema (CPFE) have been published. However, the relationship between the temporal decrease of respiratory function and the prognosis is not well known.
Methods: This study was performed to quantify the annual change in respiratory function of patients with CPFE and to compare the relationship between the change in respiratory function and survival in CPFE patients with that of patients with idiopathic pulmonary fibrosis alone (IPF-alone). Of patients who were diagnosed with IPF at discharge from our department, 32 who met the following criteria were selected as CPFE patients: 1) the presence of dyspnoea of insidious onset, 2) a computed tomography (CT) scan showing both round areas with low attenuation suggestive of emphysema and diffuse reticular opacities with sub-pleural predominance, 3) impaired ventilatory and/or diffusing capacity, and 4) an absence of known causes of pulmonar
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y fibrosis. Fifty-five patients who met the following criteria were categorized as having IPF alone: 1) a CT scan showing diffuse reticular opacities with sub-pleural predominance but no emphysema, 2) compliance with criteria 1, 3, and 4 as described for CPFE patients. The survival was compared between these 2 groups using Kaplan-Meier method. Of these patients, 26 CPFE and 33 IPF-alone patients who had undergone repeated respiratory function tests with at least one-year interval were selected for the further analysis of respiratory functions and survival.
Results: Results are expressed as medians and ranges. The baseline vital capacity (VC) of CPFE patients was greater than that of patients with IPF alone (90% [28%-132%] vs. 73% [28%-113%], respectively, p=0.018). The annual % decrease in VC from the baseline was significantly less in CPFE patients than in those with IPF alone (-3.1% [-7.8% to +10.7%] vs. -6.1% [-33.7% to +1.4%], respectively, p<0.001). Baseline diffusing-capacity (DL_co) was significantly lower in patients with CPFE than in those with IPF alone (46% [9%-75%] vs. 59.5% [15%-103%], respectively, p=0.003). DL_co/VA (transfer factor) at baseline was also lower in CPFE patients than in those with IPF alone (53% [8%-82%) vs. 76% [40%-126%], respectively, p<0.001). However, the annual decreases in DL_co and DL_co/VA in CPFE patients were modest compared with those of patients with IPF alone (DL_co: -2.4% [-16.5% to 8.1%] vs. -11.9% [-33.8% to +4.2%], respectively, p = 0064; DL_co/VA:-3.9% [-25.6% to +7.8%] vs.-3.4% [-28.0% to +15.3%], respectively, p = 0.464). Survival of 32 CPFE patients was significantly longer than that of 55 patients with IPF-alone according to Kaplan-Meier analysis (p = 0.043).
Conclusion : Well-preserved lung volume in combined pulmonary fibrosis and emphysema complicates prediction of the prognosis of CPFE. Detection of coexistent emphysema in IPF patients using CT is important to avoid making an erroneous prognosis. Delay of the temporal decrease of ventilatory and diffusing capacity needs to be further analyzed as a prognostic indicator of CPFE. Less
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