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

背景：最近，越来越多的关于肺纤维化和肺气肿(CPFE)的临床研究被发表。然而，呼吸功能的暂时性下降与预后的关系尚不清楚。方法：本研究量化CPFE患者呼吸功能的年变化，并比较CPFE患者与单纯特发性肺纤维化(IPF)患者呼吸功能变化与生存的关系。在我科出院时诊断为肺间质纤维化的患者中，32名符合以下标准的患者被选为慢性肺功能衰竭患者：1)潜伏性呼吸困难的存在，2)计算机断层扫描显示提示肺气肿的圆形低密度区和弥漫性网状阴影伴胸膜下优势，3)呼吸和/或弥散能力受损，以及4)没有已知的原因导致肺…。更多的纤维化症。符合以下标准的55名患者被分为单独的IPF患者：1)CT扫描显示弥漫性网状影，胸膜下占优势，但没有肺气肿；2)符合CPFE患者的标准1、3和4。两组生存率比较采用Kaplan-Meier法。在这些患者中，26例CPFE患者和33例IPF患者接受了至少一年的重复呼吸功能测试，用于进一步分析呼吸功能和生存情况。CPFE患者的基线肺活量(VC)高于单纯IPF患者(分别为90%[28%~132%]和73%[28%~113%]，P=0.018)。慢性阻塞性肺功能衰竭患者的肺活量较基线的年下降百分率明显低于单纯肺纤维化患者(分别为-3.1%[-7.8%至+10.7%]和-6.1%[-33.7%至+1.4%]，p&lt；0.001)。CPFE患者的基线弥散能力(DLco)显著低于单纯IPF患者(分别为46%[9%~75%]和59.5%[15%~103%]，P=0.003)。CPFE患者基线时的DLco/VA(转移因子)也低于单纯IPF患者(分别为53%[8%-82%]和76%[40%-126%]，p&lt；0.001)。但CPFE患者的Dl_co和Dl_co/VA较单纯IPF患者略有下降(Dl_co分别为-2.4%[-16.5%~8.1%]和-11.9%[-33.8%~+4.2%]，p=0064；Dl_co/VA分别为-3.9%[-25.6%~+7.8%]和-3.4%[-28.0%~+15.3%]，p=0.464)。经Kaplan-Meier分析，32例CPFE患者的生存期明显长于55例单纯IPF患者(p=0.043)。结论：肺纤维化合并肺气肿患者的肺容量保存良好使预测CPFE的预后变得困难。CT检查肺间质纤维化合并肺气肿对避免错误预后有重要意义。通气量和弥散量暂时性下降的延迟需要进一步分析，以此作为CPFE的预后指标。较少

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