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

背景：最近，越来越多的临床研究肺纤维化和肺气肿（CPFE）已公布。然而，呼吸功能的时间减少和预后之间的关系是不为人所知的方法：本研究进行量化的CPFE患者的呼吸功能的年度变化，并比较在CPFE患者的呼吸功能和生存的变化之间的关系与特发性肺纤维化患者（IPF单独）。在我科出院时诊断为IPF的患者中，32例符合以下标准的患者被选为CPFE患者：1）存在隐匿性发作的呼吸困难，2）计算机断层扫描（CT）显示圆形区域（低衰减，提示肺气肿）和弥漫性网状阴影（胸膜下占优势），3）呼吸和/或弥散能力受损，和4）缺乏已知的肺动脉炎原因 关于我们 y纤维化。符合以下标准的55例患者被归类为单独患有IPF：1）CT扫描显示弥漫性网状阴影伴胸膜下优势，但无肺气肿，2）符合CPFE患者描述的标准1、3和4。采用Kaplan-Meier法比较两组患者的生存率。在这些患者中，26例CPFE和33例IPF单独患者进行了重复的呼吸功能测试，至少间隔一年，被选为进一步分析的呼吸功能和survival.Results：结果表示为中位数和范围。CPFE患者的基线肺活量（VC）大于单纯IPF患者（分别为90% [28%-132%] vs. 73% [28%-113%]，p=0.018）。CPFE患者VC相对于基线的年下降百分比显著低于单纯IPF患者（分别为-3.1% [-7.8%至+10.7%] vs. -6.1% [-33.7%至+1.4%]，p<0.001）。CPFE患者的基线弥散能力（DL_co）显著低于单纯IPF患者（分别为46% [9%-75%] vs. 59.5% [15%-103%]，p=0.003）。CPFE患者的基线DL_co/VA（转移因子）也低于单纯IPF患者（分别为53% [8%-82%] vs. 76% [40%-126%]，p<0.001）。然而，与单纯IPF患者相比，CPFE患者的DL_co和DL_co/VA的年下降幅度较小（DL_co：-2.4% [-16.5%至8.1%] vs. -11.9% [-33.8%至+4.2%]，p = 0064; DL_co/VA：-3.9% [-25.6%至+7.8%] vs.- 3.4% [-28.0%至+15.3%]，p = 0.464）。Kaplan-Meier分析显示，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肺容积保存完好，可作为预测CPFE预后的重要依据。使用CT检测IPF患者中共存的肺气肿对于避免做出错误的预后很重要。作为CPFE的预后指标，需要进一步分析延迟的释光和弥散能力的时间下降。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