Wells Athol U, Desai Sujal R, Rubens Michael B, Goh Nicole S L, Cramer Derek, Nicholson Andrew G, Colby Thomas V, du Bois Roland M, Hansell David M
Department of Radiology, Royal Brompton Hospital, Interstitial Lung Disease Unit, Emmanuel Kaye Building, Manresa Road, Chelsea, London SW6 LR6, UK.
Am J Respir Crit Care Med. 2003 Apr 1;167(7):962-9. doi: 10.1164/rccm.2111053.
In idiopathic pulmonary fibrosis, the quantitation of disease severity using pulmonary function tests is often confounded by emphysema. We have identified the composite physiologic index (CPI) most closely reflecting the morphologic extent of pulmonary fibrosis. Consecutive patients with a clinical/computed tomography (CT) diagnosis of idiopathic pulmonary fibrosis (n = 212) were divided into group I (n = 106) and group II (n = 106). The CPI was derived in group I (by fitting pulmonary function tests against disease extent on CT) and was tested in Group II. The formula for the CPI was as follows: extent of disease on CT = 91.0 - (0.65 x percent predicted diffusing capacity for carbon monoxide [DLCO]) - (0.53 x percent predicted FVC) + (0.34 x percent predicted FEV1). In group II, the CPI correlated more strongly with disease extent on CT (r2 = 0.51) than the individual pulmonary function test (DLCO the highest value, r2 = 0.38). A subanalysis demonstrated that the better fit of the CPI was ascribable to a correction of the confounding effects of emphysema. Mortality was predicted more accurately by the CPI than by a pulmonary function test in all clinical subgroups, including a separate cohort of 36 patients with histologically proven usual interstitial pneumonia (CPI, p < 0.0005; FVC, p = 0.002; PO2, p = 0.002). In conclusion, a new CPI, derived against CT and validated using split sample testing, is a more accurate prognostic determinant in usual interstitial pneumonia than an individual pulmonary function test.
在特发性肺纤维化中,使用肺功能测试来量化疾病严重程度常常因肺气肿而变得复杂。我们已经确定了最能反映肺纤维化形态学范围的综合生理指数(CPI)。将连续的临床/计算机断层扫描(CT)诊断为特发性肺纤维化的患者(n = 212)分为I组(n = 106)和II组(n = 106)。在I组中得出CPI(通过将肺功能测试与CT上的疾病范围进行拟合),并在II组中进行测试。CPI的公式如下:CT上的疾病范围 = 91.0 - (0.65×预测的一氧化碳弥散能力百分比[DLCO]) - (0.53×预测的用力肺活量百分比[FVC]) + (0.34×预测的第一秒用力呼气量百分比[FEV1])。在II组中,CPI与CT上的疾病范围的相关性(r2 = 0.51)比单个肺功能测试更强(DLCO相关性最高,r2 = 0.38)。一项亚分析表明,CPI的更好拟合归因于对肺气肿混杂效应的校正。在所有临床亚组中,包括一组36例经组织学证实为普通型间质性肺炎的患者,CPI比肺功能测试更准确地预测死亡率(CPI,p < 0.0005;FVC,p = 0.002;PO2,p = 0.002)。总之,一种根据CT得出并经拆分样本测试验证的新CPI,在普通型间质性肺炎中是比单个肺功能测试更准确的预后决定因素。