Sun D, Cheng Z, Jiang T C, Li P F, Jia L Q, Wang T T, Zheng C P, Li Y, Duo M J
Department of Respiratory and Critical Care Medicine, the First Affiliated Hospital of Zhengzhou University; Institute of Clinical Medical Research of Universities of Henan; Henan Key Laboratory for Pharmacology of Liver Diseases, Zhengzhou 450052, China.
Zhonghua Yi Xue Za Zhi. 2020 Mar 17;100(10):748-752. doi: 10.3760/cma.j.cn112137-20191008-02166.
To study the characteristics and clinical significance of pulmonary function test and kerbs von den lungen 6 (KL-6) in anti-synthetase syndrome related interstitial lung disease (ASSD-ILD) and idiopathic pulmonary fibrosis (IPF). The clinical data of 43 patients with ASSD-ILD (ASSD-ILD group) from May 2015 to May 2017 were collected retrospectively, including 12 males and 31 females, and 34 patients with IPF (IPF group) treated in the First Affiliated Hospital of Zhengzhou University during the same period, including 28 males and 6 females, were also included. The basic information, and the value of pulmonary function test [pulmonary function parameters included the forced vital capacity expressed as percent predicted (FVC%pred), the forced expiratory volume in 1 second expressed as percent predicted (FEV(1)%pred), the ratio of FVC to FEV(1) (FVC/FEV(1)), the peak expiratory flow expressed as percent predicted (PEF%pred), the forced expiratory flow at 25%, 50%, 75% of FVC as percent predicted (FEF(25)%pred, FEF(50)%pred, and FEF(75)%pred), the maximum mid-expiratory flow as percent predicted (MMEF% pred), and the diffusing capacity for carbon monoxide as percent predicted (DLCO% pred)], and serum KL-6 level in ASSD-ILD and IPF were compared. The FEV(1)%pred, FEF(50)%pred, FEF(75)%pred, and MMEF%pred values in ASSD-ILD group were significantly lower than those in IPF group (all 0.05), while the FVC% pred, FVC/FEV(1), PEF% pred, FEF(25)%pred, and DLCO% pred values in ASSD-ILD group had no significant difference compared with IPF group (all 0.05). There was no significant difference in serum KL-6 level between ASSD-ILD group and IPF group [(1 169±911) vs (1 210±908) U/ml, =0.62, =0.463]. Follow-up analysis showed that the serum KL-6 level of ASSD-ILD patients who died within two years was significantly higher than that of survivors [(2 060±1 168) vs (1 042±858) U/ml, =2.93, =0.041]. The serum KL-6 level of patients who died within two years of IPF patients was also significantly higher than that of patients who survived [(1 767±865) vs (1 089±894) U/ml, =2.53, =0.026]. The serum KL-6 level in ASSD-ILD group was negatively correlated with FVC%pred (=-0.43, =0.004), FEV(1)%pred (=-0.39, =0.010) and DLCO% pred (=-0.41, =0.006). There was no correlation between serum KL-6 level and pulmonary function test indexes in IPF group (all 0.05). There is difference in pulmonary function test between ASSD-ILD patients and IPF patients. High serum KL-6 level will be predictive of poor prognosis.
研究肺功能测试及克拉布肺6(KL-6)在抗合成酶综合征相关间质性肺病(ASSD-ILD)和特发性肺纤维化(IPF)中的特征及临床意义。回顾性收集2015年5月至2017年5月43例ASSD-ILD患者(ASSD-ILD组)的临床资料,其中男性12例,女性31例,同期纳入郑州大学第一附属医院收治的34例IPF患者(IPF组),其中男性28例,女性6例。比较两组患者的基本信息、肺功能测试值[肺功能参数包括以预测值百分比表示的用力肺活量(FVC%pred)、以预测值百分比表示的第1秒用力呼气容积(FEV(1)%pred)、FVC与FEV(1)的比值(FVC/FEV(1))、以预测值百分比表示的呼气峰值流速(PEF%pred)、以预测值百分比表示的FVC 25%、50%、75%时的用力呼气流量(FEF(25)%pred、FEF(50)%pred和FEF(75)%pred)、以预测值百分比表示的最大呼气中期流速(MMEF% pred)以及以预测值百分比表示的一氧化碳弥散量(DLCO% pred)]及血清KL-6水平。ASSD-ILD组的FEV(1)%pred、FEF(50)%pred、FEF(75)%pred和MMEF%pred值显著低于IPF组(均P<0.05),而ASSD-ILD组的FVC% pred、FVC/FEV(1)、PEF% pred、FEF(25)%pred和DLCO% pred值与IPF组相比差异无统计学意义(均P>0.05)。ASSD-ILD组与IPF组血清KL-6水平差异无统计学意义[(1 169±911)vs(1 210±908)U/ml,t=0.62,P=0.463]。随访分析显示,ASSD-ILD组2年内死亡患者的血清KL-6水平显著高于存活患者[(2 060±1 168)vs(1 042±858)U/ml,t=2.93,P=0.041]。IPF组2年内死亡患者的血清KL-6水平也显著高于存活患者[(1 767±865)vs(1 089±894)U/ml,t=2.53,P=0.026]。ASSD-ILD组血清KL-6水平与FVC%pred(r=-0.43,P=0.004)、FEV(1)%pred(r=-0.39,P=0.010)及DLCO% pred(r=-0.41,P=0.006)呈负相关。IPF组血清KL-6水平与肺功能测试指标无相关性(均P>0.05)。ASSD-ILD患者与IPF患者的肺功能测试存在差异。血清KL-6水平升高提示预后不良。