THE DIAGNOSIS AND THE TREATMENT BY STEROIDS IN DIOPATHIC INTERSTITIAL PNEUMONIAS BASED ON THE TRANSBRONHIAL LUNG BIOPSY

基于经支气管肺活检的特发性间质性肺炎的诊断和类固醇治疗

基本信息

批准号：
16590770
负责人：
WATANABE Kentaro
金额：
$ 2.08万
依托单位：
Fukuoka University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (C)
财政年份：
2004
资助国家：
日本
起止时间：
2004 至 2007
项目状态：
已结题

项目摘要

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 … More 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

背景：最近，越来越多的肺纤维化合并肺气肿（CPFE）的临床研究被发表，然而，呼吸功能暂时下降与预后之间的关系尚不清楚。 CPFE 患者呼吸功能的年度变化，并比较 CPFE 患者与单纯特发性肺纤维化（IPF-单纯）诊断患者的呼吸功能变化与生存之间的关系。我们科室出院时患有 IPF，符合以下标准的 32 名患者被选为 CPFE 患者：1) 存在隐匿性呼吸困难，2) 计算机断层扫描 (CT) 扫描显示两个圆形区域具有低衰减，提示存在肺气肿和弥漫性网状混浊，以胸膜下为主，3）通气和/或弥散能力受损，4）缺乏已知的肺...符合以下标准的 55 名患者被归类为仅患有 IPF：1) CT 扫描显示弥漫性网状混浊，以胸膜下为主，但无肺气肿，2) 符合标准 1、3 和4 如 CPFE 患者所述，使用 Kaplan-Meier 方法比较这 2 组患者的生存率，其中 26 名 CPFE 患者和 33 名单独使用 IPF 的患者。选择间隔至少一年进行重复呼吸功能测试的患者来进一步分析呼吸功能和生存情况。结果：结果以中位数和范围表示，CPFE 患者的基线肺活量（VC）大于 CPFE 患者的基线肺活量（VC）。仅患有 IPF 的患者（分别为 90% [28%-132%] 与 73% [28%-113%]，p=0.018）VC 相对于基线的年下降百分比显着减少。 CPFE 患者的死亡率高于仅患有 IPF 的患者（分别为 -3.1% [-7.8% 至 +10.7%] 与 -6.1% [-33.7% 至 +1.4%]，p<0.001）。）在 CPFE 患者中显着低于仅患有 IPF 的患者（46% [9%-75%] vs. 59.5%） [15%-103%]，p=0.003，CPFE 患者基线时的 DL_co/VA（转移因子）也低于仅患有 IPF 的患者（53% [8%-82%] vs. 76%）。 [40%-126%]，p<0.001）然而，与那些患者相比，CPFE 患者的 DL_co 和 DL_co/VA 的年度下降幅度不大。单纯 IPF 患者的比例（DL_co：分别为 -2.4% [-16.5% 至 8.1%] 与 -11.9% [-33.8% 至 +4.2%]，p = 0064；DL_co/VA：-3.9% [-25.6 % 至 +7.8%] 与-3.4% [-28.0% 至 +15.3%] 分别，p =根据 Kaplan-Meier 分析，32 名 CPFE 患者的生存期明显长于 55 名仅接受 IPF 的患者（p = 0.043）。结论：肺纤维化和肺气肿合并时肺容量保存完好，使预后预测变得复杂。使用 CT 检测 IPF 患者共存的肺气肿对于避免做出错误的预后非常重要。通气和弥散能力暂时下降的延迟需要作为 CPFE 的预后指标进行进一步分析。