Department of Pulmonary Medicine, Kameda Medical Center, Kamogawa, Japan.
Department of Immunology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo Ward, Chiba, Japan.
Respir Res. 2020 Oct 20;21(1):275. doi: 10.1186/s12931-020-01545-3.
Combined pulmonary fibrosis and emphysema (CPFE) is a heterogeneous clinico-radiological syndrome without a consensus definition. There are limited data on the relation between the amount of parenchymal fibrosis and prognosis. In this study, we assessed the prognostic implications of the extent of fibrosis assessed by an automated quantitative computed tomography (CT) technique and the radiological and functional change over time in patients with a broad spectrum of fibrotic interstitial lung diseases (ILDs) encountered in a real-world setting.
We conducted a single-centre, retrospective study of 228 consecutive patients with CPFE, encountered from 2007 to 2015 at Kameda Medical Center, Chiba, Japan. We investigated the prognostic value of automated CT fibrosis quantification and the subsequent course of CPFE.
Among 228 patients with CPFE, 89 had fibrosis affecting < 5% of their lungs, 54 had 5 to < 10% fibrosis, and 85 had ≥ 10% fibrosis at the time of diagnosis. Lower volume of fibrosis correlated with lower rates of mortality and acute exacerbation (p < 0.001). In particular, among those with < 5% fibrosis, only 4.5% died and none experienced acute exacerbation during follow-up, whereas 57.6% and 29.4% of those with ≥ 10% fibrosis experienced death and acute exacerbation, respectively. Although, the ≥ 10% fibrosis group had the poorest overall survival as well as the highest incidence of acute exacerbation, the incidence of decline in pulmonary function tests, change per year in total lung volume, and progression of fibrosis on chest CT was highest in the 5 to < 10% fibrosis group. The Cox proportional hazard model for CPFE progression (defined by composite criteria of death, acute exacerbation, and decline in forced vital capacity or diffusing capacity) showed fibrosis proportion was a risk factor independent of age, sex, smoking pack-years, the Charlson Comorbidity Index, lung cancer, connective tissue disease, and idiopathic pulmonary fibrosis.
Less severe (< 5%) fibrosis at baseline was associated with disease stability and better prognosis compared to more severe fibrosis in CPFE occurring with fibrotic ILDs. Further studies including a validation cohort will be needed. Trial Registration Retrospectively registered.
合并性肺纤维化和肺气肿（CPFE）是一种临床-影像学表现异质性综合征，目前尚无共识定义。关于实质纤维化程度与预后之间的关系，相关数据有限。本研究通过一种自动化定量计算机断层扫描（CT）技术评估了纤维化程度，并评估了在现实环境中遇到的各种纤维化间质性肺疾病（ILDs）患者的纤维化程度随时间推移的变化，以此来评估其预后意义。
我们进行了一项单中心、回顾性研究，纳入了 2007 年至 2015 年期间在日本千叶县镰仓医疗中心就诊的 228 例 CPFE 连续患者。我们调查了自动化 CT 纤维化定量检测的预后价值以及 CPFE 的后续病程。
在 228 例 CPFE 患者中，89 例患者的肺部纤维化程度<5%，54 例患者的肺部纤维化程度为 5%至<10%，85 例患者的肺部纤维化程度≥10%。较低的纤维化程度与死亡率和急性加重率较低相关（p<0.001）。特别是在纤维化程度<5%的患者中，只有 4.5%的患者死亡，没有患者在随访期间发生急性加重，而纤维化程度≥10%的患者中，分别有 57.6%和 29.4%的患者死亡和发生急性加重。尽管≥10%纤维化组的总体生存率最差，急性加重发生率最高，但在肺功能测试下降、总肺容积每年变化和胸部 CT 上纤维化进展方面，5%至<10%纤维化组的发生率最高。CPFE 进展的 Cox 比例风险模型（通过死亡、急性加重和用力肺活量或弥散量下降的综合标准定义）显示，纤维化比例是一个独立于年龄、性别、吸烟包年数、Charlson 合并症指数、肺癌、结缔组织病和特发性肺纤维化的危险因素。
与 CPFE 中发生的纤维化间质性肺疾病相比，基线时较轻的（<5%）纤维化与疾病稳定和更好的预后相关。还需要进一步的包括验证队列的研究。
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