Zompatori M, Fasano L, Battista G, Cavina M, Bertaccini P
Policlinico S. Orsola-Malpighi, Bologna.
Radiol Med. 1997 Dec;94(6):611-7.
We studied the HRCT and functional evolution of idiopathic pulmonary fibrosis (IPF) patients presenting with Wells grade III--prevalent fibrosis.
We sequentially studied the HRCT and functional findings of 16 IPF patients, at presentation and at 1 year. All patients had a typical grade III IPF pattern; those with the most severe clinical presentation were treated (9/16). The main HRCT parameters were the extent of interstitial involvement and emphysema (visual score) and the mean diameter of lung cysts in honeycombing regions.
Most of our grade III IPF patients exhibited a slowly progressive deterioration, with no accelerated parenchymal opacification. Deterioration was found on HRCT images in 56.2% of patients (p = .02), with a mean monthly increase of .56%. Fibrosis extent, evaluated as HRCT visual score at presentation, was significantly correlated with viability and PaO2, values (p = .01). Follow-up HRCT scores were also significantly correlated with viability (p = .004). The mean diameter of honeycomb lung cysts increased in 25% of patients. Emphysema was associated at presentation in 50% of patients--all of them former smokers; it was less diffuse than interstitial involvement (15% of total lung volume versus 46.7% at presentation) and was not seen to progress on follow-up images. The comparison between treated (T) and untreated (NT) patients confirmed more severe HRCT and functional damage in T patients at presentation. Moreover, T patients presented a significantly more rapid deterioration, despite treatment, than NT patients, who had less severe and slower HRCT and functional evolution, excluding DLCO deterioration (p = .01). To conclude, grade III IPF patients can be subdivided into two subgroups, with rather different prognosis and evolution, on the basis of HRCT and functional findings at presentation. The current treatment seems useless in grade III IPF. HRCT findings, integrated with the visual score of disease extent, and lung function tests can be used to monitor grade III IPF evolution.
我们研究了表现为韦尔斯III级（普遍存在纤维化）的特发性肺纤维化（IPF）患者的高分辨率计算机断层扫描（HRCT）及功能演变情况。
我们对16例IPF患者在初诊时及1年后的HRCT和功能检查结果进行了连续研究。所有患者均具有典型的III级IPF模式；其中临床表现最严重的患者接受了治疗（9/16）。主要的HRCT参数包括间质受累程度和肺气肿（视觉评分）以及蜂窝状区域肺囊肿的平均直径。
我们的大多数III级IPF患者表现出缓慢进展的恶化，没有实质性混浊加速的情况。56.2%的患者在HRCT图像上出现了恶化（p = 0.02），平均每月增加0.56%。初诊时作为HRCT视觉评分评估的纤维化程度与生存能力和动脉血氧分压（PaO2）值显著相关（p = 0.01）。随访时的HRCT评分也与生存能力显著相关（p = 0.004）。25%的患者蜂窝状肺囊肿平均直径增大。50%的患者初诊时存在肺气肿，他们均为既往吸烟者；肺气肿的范围比间质受累范围小（占肺总体积的15%，而初诊时为46.7%），且在随访图像上未见进展。治疗组（T）和未治疗组（NT）患者的比较证实，T组患者初诊时HRCT和功能损害更严重。此外，尽管接受了治疗，但T组患者的恶化速度明显快于NT组患者，NT组患者的HRCT和功能演变程度较轻且较慢，不包括一氧化碳弥散量（DLCO）恶化（p = 0.01）。总之，根据初诊时的HRCT和功能检查结果，III级IPF患者可分为两个亚组，其预后和演变情况差异较大。目前的治疗方法对III级IPF似乎无效。结合疾病范围视觉评分的HRCT检查结果和肺功能测试可用于监测III级IPF的演变。
