Objective: 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). Methods: 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. Results: 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 P<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 P>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, t=0.62, P=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, t=2.93, P=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, t=2.53, P=0.026]. The serum KL-6 level in ASSD-ILD group was negatively correlated with FVC%pred (r=-0.43, P=0.004), FEV(1)%pred (r=-0.39, P=0.010) and DLCO% pred (r=-0.41, P=0.006). There was no correlation between serum KL-6 level and pulmonary function test indexes in IPF group (all P>0.05). Conclusions: 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.
目的： 探讨肺功能和涎液化糖链抗原-6（KL-6）在抗合成酶综合征合并间质性肺疾病（ASSD-ILD）与特发性肺纤维化（IPF）中的特点及临床意义。 方法： 回顾性收集2015年5月至2017年5月郑州大学第一附属医院诊治的43例ASSD-ILD患者（其中男12例，女31例）（ASSD-ILD组）及34例IPF患者（其中男28例，女6例）（IPF组）的临床资料，比较两组患者基本信息、肺功能结果[包括用力肺活量占预计值的百分比（FVC%预计值）、第1秒用力呼气容积占预计值的百分比（FEV(1)%预计值）、FEV(1)与FVC的比值（FEV(1)/FVC）、呼气峰值流量占预计值的百分比（PEF%预计值）、用力呼出25%、50%、75%肺活量时的瞬间呼气流量占预计值的百分比（FEF(25)%预计值、FEF(50)%预计值、FEF(75)%预计值）、最大呼气中期流量占预计值的百分比（MMEF%预计值）、肺弥散量占预计值的百分比（DLCO%预计值）]及KL-6水平，分析ASSD-ILD与IPF组中肺功能和KL-6的特点及临床意义。 结果： ASSD-ILD组FEV(1)%预计值、FEF(50)%预计值、FEF(75)%预计值、MMEF%预计值均显著低于IPF组（均P<0.05），而ASSD-ILD组FVC%预计值、FEV(1)/FVC、PEF%预计值、FEF(25)%预计值及DLCO%预计值与IPF组相比差异无统计学意义（均P>0.05）。ASSD-ILD组血清KL-6水平与IPF组相比差异无统计学意义[（1 169±911）比（1 210±908）U/ml，t=0.62，P=0.463]。随访分析表明，ASSD-ILD两年内死亡患者血清KL-6水平显著高于存活患者[（2 060±1 168）比（1 042±858）U/ml，t=2.93，P=0.041]；IPF两年内死亡患者血清KL-6水平也显著高于存活患者[（1 767±865）比（1 089±894）U/ml，t=2.53，P=0.026]。ASSD-ILD组血清KL-6水平与FVC%预计值（r=-0.43，P=0.004）、FEV(1)%预计值（r=-0.39，P=0.010）及DLCO%预计值（r=-0.41，P=0.006）均呈负相关；IPF组血清KL-6水平与肺功能各项指标均无相关性（均P>0.05）。 结论： ASSD-ILD患者与IPF患者之间肺功能存在差异，高水平血清KL-6提示患者预后较差。.
Keywords: Anti-synthetase syndrome; Kerbs von den lungen 6; Lung disease, interstitial; Pulmonary fibrosis; Pulmonary function test.