Nakamura Yasuhiko, Sugino Keishi, Kitani Masashi, Hebisawa Akira, Tochigi Naobumi, Homma Sakae
Department of Respiratory Medicine, Toho University Omori Medical Center, Japan.
Department of Respiratory Medicine, Toho University Omori Medical Center, Japan.
Respir Investig. 2018 Jan;56(1):40-47. doi: 10.1016/j.resinv.2017.09.001. Epub 2017 Oct 21.
The purpose of this study was to clarify the clinico-radio-pathological characteristics and prognostic factors of unclassifiable-idiopathic interstitial pneumonias (U-IIPs) diagnosed by surgical lung biopsy.
Among 86 patients with interstitial pneumonia who underwent surgical lung biopsy from January 2005 to September 2013, 33 (38.4%; 16 male patients; mean age, 64.4 ± 8.8 years) were diagnosed with U-IIPs. They were subsequently categorized into rapidly progressive (n = 7), slowly progressive (n = 7), and stable (n = 19) groups based on the decrease of the percent predicted forced vital capacity or percent predicted diffusing capacity of the lung carbon monoxide and the occurrence of acute exacerbation. The clinico-radio-pathological features and survival rates of the patients who were followed up for at least 3 years were examined. These cases were reevaluated retrospectively by multidisciplinary discussion.
The rapidly progressive group had a significantly poorer prognosis than that of the other groups (p < 0.0001). Although there were no significant pattern differences on the chest high-resolution computed tomography, the fibrosis scores were significantly higher in the rapidly progressive group (p = 0.002). Furthermore, the percentage of fibroblastic foci assessed by the pathological analysis was also significantly higher in the rapidly progressive group (p = 0.006). Nine (27.3%) patients developed connective tissue diseases during follow-up.
The radiologic patterns were not significantly different among the three clinical U-IIPs subgroups. Nevertheless, our findings suggested that the fibrosis scores and the percentage of fibroblastic foci could provide a prognostic assessment in U-IIPs.
本研究旨在阐明经外科肺活检诊断的不可分类的特发性间质性肺炎（U-IIP）的临床-放射-病理特征及预后因素。
2005年1月至2013年9月间，对86例行外科肺活检的间质性肺炎患者进行研究，其中33例（38.4%；男性16例；平均年龄64.4±8.8岁）被诊断为U-IIP。随后根据预测的用力肺活量百分比或预测的肺一氧化碳弥散量的下降情况以及急性加重的发生情况，将他们分为快速进展组（n = 7）、缓慢进展组（n = 7）和稳定组（n = 19）。对随访至少3年的患者的临床-放射-病理特征及生存率进行了检查。通过多学科讨论对这些病例进行回顾性重新评估。
快速进展组的预后明显比其他组差（p < 0.0001）。尽管胸部高分辨率计算机断层扫描上没有显著的模式差异，但快速进展组的纤维化评分明显更高（p = 0.002）。此外，病理分析评估的成纤维细胞灶百分比在快速进展组中也明显更高（p = 0.006）。9例（27.3%）患者在随访期间发生了结缔组织病。
三种临床U-IIP亚组的放射学模式没有显著差异。然而，我们的研究结果表明，纤维化评分和成纤维细胞灶百分比可为U-IIP提供预后评估。
