纤维化性间质性肺疾病预后因素的前瞻性队列研究

Prognostic factors for fibrosing interstitial lung diseases:a prospective cohort study

摘要:

目的 探讨纤维化性间质性肺疾病的临床特点和发展为进行性纤维化型及死亡的风险因素。方法 选择2019年5月至12月解放军总医院呼吸科收治的58例纤维化性间质性肺疾病患者为研究对象。收集临床资料,行肺CT、肺功能等检查,同时行6min步行试验,应用加利福尼亚大学圣地亚哥分校呼吸困难问卷(SOBQ)进行呼吸困难评分,并随访至1年进行病情评估。应用SPSS 19.0软件和R 3.6.1软件进行统计分析。根据数据类型,分别采用t检验或χ²检验进行组间比较。采用竞争风险模型的生存分析和Fine-Gray回归模型提取发展为进行性纤维化型和死亡风险的影响因素。结果 58例患者中,35例(60.34%)诊断为特发性肺纤维化(IPF),14例(24.14%)诊断为结缔组织病相关间质性肺病,9例(15.52%)归为其他原因所致的肺纤维化；12例(20.69%)患者发展为进行性纤维化型,8例(13.79%)患者死亡(6例死于纤维化急性加重)。单因素分析结果显示,发展为进行性纤维化型的风险因素是诊断为IPF、高分辨CT(HRCT)网格影评分和蜂窝影评分高,死亡的风险因素是6min步行距离短(＜300m的患者死亡率明显增高)、SOBQ评分高。多因素分析结果显示,HRCT网格影评分高是发展为进行性纤维化型的风险因素(RC=0.687,HR=1.99,95%CI 1.03~3.85,P=0.042),未发现死亡的风险因素。结论 诊断为IPF、HRCT网格影和蜂窝影评分高的患者发展为进行性纤维化型的风险更高；6min步行距离＜300m、SOBQ评分高的患者死亡风险更高。

Abstract:

Objective To investigate the clinical characteristics of fibrosing interstitial lung diseases, and explore its risk factors of mortality and of developing a progressive phenotype. Methods A total of 58 patients who suffered from fibrosing interstitial lung diseases admitted to the Chinese PLA General Hospital during May and December 2019 were prospectively enrolled in this study. Their clinical data were collected. And chest CT scanning, lung function test and 6-minute walk test(6MWT) were performed. The dyspnea score was assessed with the University of California, San Diego shortness of breath questionnaire (SOBQ). The patients were followed up for 1 year to evaluate the condition. SPSS statistics 19.0 and R 3.6.1 were used to perform the statistical analysis. Student′s t test or Chi-square test was employed for intergroup comparison for different data types. Competing risk model and Fine-Gray competing risk regression model were adopted to extract the risk factors of progressive fibrosis and mortality. Results Of the 58 patients, 35 (60.34%) were diagnosed as idiopathic pulmonary fibrosis (IPF), 14 (24.14%) as interstitial lung disease associated with connective tissue diseases, and 9 (15.52%) as pulmonary fibrosis caused by other causes. Twelve cases (20.69%) developed into progressive fibrotic phenotype, and 8 (13.79%) died, including 6 due to acute exacerbation of fibrosis. Univariate analysis showed that the risk factors for the development of progressive fibrotic phenotype were diagnosis of IPF, high reticular score and honeycombing score in high resolution CT (HRCT) scans, and the risk factors for death were short 6-minute walk distance (the dead had a significantly higher ratio of shorter than 300 m) and higher SOBQ score. Multivariate analysis showed that high HRCT reticular score was the risk factor for progressive fibrotic phenotype (RC=0.687, HR=1.99,95%CI 1.03-3.85, P=0.042), and no risk factors for death were found. Conclusion Patients with IPF, high HRCT reticular and honeycombing scores are at a higher risk of developing progressive fibrotic phenotype. Those with 6-minute walk distance shorter than 300 m and higher SOBQ scores are prone to death.