硬皮病相关间质性肺病与特发性肺纤维化的临床比较

Clinical Comparison Between Scleroderma Associated Interstitial Lung Disease and Idiopathic Pulmonary Fibrosis

目的比较系统性硬化症相关肺间质性病变(SSc-ILD)与特发性肺纤维化(IPF)患者的临床特点。方法收集甘肃省人民医院2018年10月-2021年11月明确诊断为SSc-ILD和IPF患者资料,对其临床特点进行回顾性分析,并采用Praeson线性相关分析乳酸脱氢酶水平与DLCOpre%及HRCT评分的相关性,采用多因素线性回归分析影响患者肺纤维化程度的相关因素。结果共纳入68例患者,其中SSc-ILD患者28例,IPF患者40例,SSc-ILD组的发病年龄低于IPF组,差异有统计学意义(P<0.05);SSc-ILD组多为女性,皮肤关节症状为首发表现较多,IPF组吸烟及男性患者占比较多,呼吸系统症状出现时间长,以呼吸症状为首发表现,咳嗽、咳痰、气短、乳酸脱氢酶升高,DLCO%pre降低占比高于SSc-ILD组,差异有统计学意义(P<0.05);IPF组HRCT以UIP型多见,蜂窝状影、肺纤维化评分高于SSc-ILD组,差异有统计学意义(P<0.05);SSc-ILD组HRCT以NSIP多见,纤维条索影、心包积液患者多于IPF组,差异有统计学意义(P<0.05);Praeson线性相关分析显示,LDH水平与HRCT评分呈正相关(r=0.647,P<0.05),LDH水平与DLco%pre值呈负相关(r=-0.422,P<0.05);多因素线性回归分析显示,LDH水平是肺纤维化不良预后的独立危险因素(P<0.05)。结论SSc-ILD和IPF患者的临床特点存在显著差异,自身抗体、肺功能、HRCT在肺间质性疾病的诊断中发挥重要价值,乳酸脱氢水平与SSC-ILD患者肺纤维化严重程度高度相关,临床值得注意。

Objective To compare the clinical characteristics of patients with systemic sclerosis-associated interstitial lung disease(SSc-ILD)and idiopathic pulmonary fibrosis(IPF).Methods The data of patients diagnosed with SSc-ILD and IPF in Gansu Provincial People's Hospital from October 2018 to November 2021 were collected,and their clinical characteristics were retrospectively analyzed.The correlation between lactate dehydrogenase level and DLCOpre%and HRCT score was analyzed by Praeson linear correlation.Multivariate linear regression analysis was used to analyze the related factors affecting the degree of pulmonary fibrosis in patients.Results A total of 68 patients were included,including 28 patients with SSc-ILD and 40 patients with IPF.The age of onset in the SSc-ILD group was lower than that in the IPF group,and the difference was statistically significant(P<0.05).The SSc-ILD group was mostly female,with more skin and joint symptoms as the first manifestation.In the IPF group,smoking and male patients accounted for more,respiratory symptoms appeared for a long time,with respiratory symptoms as the first manifestation,cough,sputum,shortness of breath,lactate dehydrogenase increased,DLCO%pre decreased higher than the SSc-ILD group,the difference was statistically significant(P<0.05);the HRCT of IPF group was more common in UIP type,and the scores of honeycomb shadow and pulmonary fibrosis were higher than those of SSc-ILD group(P<0.05).In SSc-ILD group,NSIP was more common in HRCT,and patients with fibrous cord shadow and pericardial effusion were more than those in IPF group,the difference was statistically significant(P<0.05).Praeson linear correlation analysis showed that LDH level was positively correlated with HRCT score(r=0.647,P<0.05),and LDH level was negatively correlated with DLCO%pre(r=-0.422,P<0.05).Multivariate linear regression analysis showed that LDH level was an independent risk factor for poor prognosis of pulmonary fibrosis(P<0.05).Conclusion There are significant differences in clinical characteristics between SSc-ILD and IPF patients.Autoantibodies,lung function and HRCT play an important role in the diagnosis of interstitial lung disease.Lactate dehydrogenation level is highly correlated with the severity of pulmonary fibrosis in SSC-ILD patients,which is worthy of clinical attention.