特发性肺纤维化与髓过氧化物酶-抗中性粒细胞胞质抗体相关血管炎相关肺纤维化的临床特征比较

Comparison of clinical characteristics in patients with idiopathic pulmonary fibrosis and myeloperoxidase-antineutrophil cytoplasmic antibody associated vasculitis associated pulmonary fibrosis

目的比较特发性肺纤维化(IPF)与髓过氧化物酶-抗中性粒细胞胞质抗体相关血管炎(MPO-AAV)相关肺纤维化患者的临床特征,以利于早期甄别MPO-AAV相关肺纤维化。方法收集首都医科大学附属北京友谊医院2017年1月1日至2022年1月1日住院的IPF患者46例及MPO-AAV相关肺纤维化患者28例,胸部高分辨率CT(HRCT)表现为普通型间质性肺炎(UIP)或可能UIP,回顾性分析两组患者基线资料、临床表现、实验室检查、血气分析、肺功能等结果。结果IPF组患者年龄、吸烟指数、咳嗽、咳痰比例均高于MPO-AAV组,MPO-AAV组发热、消瘦、关节疼痛比例高于IPF组;与IPF组患者相比,MPO-AAV组血白细胞计数、中性粒细胞百分比明显升高,血红蛋白明显降低,血小板、血C反应蛋白、红细胞沉降率、尿素氮、肌酐明显升高,尿潜血及尿蛋白阳性率明显升高;IPF组较MPO-AAV组患者血乳酸脱氢酶、癌胚抗原、细胞角蛋白19片段抗原升高;MPO-AAV组与IPF组相比,动脉血pH升高,动脉血二氧化碳分压、血碳酸氢根降低;IPF组较MPO-AAV组用力肺活量(FVC)、一氧化碳弥散量占预计值百分比(DLCO%pred)降低,以上差异均有统计学意义(P<0.05)。结论对于胸部HRCT表现为UIP或可能UIP的患者,如患者有全身症状(发热、消瘦、关节疼痛)、红细胞沉降率快、贫血及肾损害情况,应考虑MPO-AAV可能。已经诊断为IPF的患者,如随访过程中出现上述表现,也应及时复查自身抗体以警惕MPO-AAV。

Objective To enhance the recognition of pulmonary fibrosis caused by myeloperoxidase-antineutrophil cytoplasmic antibody associated vasculitis(MPO-AAV)by comparing the clinical characteristics in patients with idiopathic pulmonary fibrosis(IPF)and MPO-AAV associated pulmonary fibrosis.Methods Collect the data of 46 patients with IPF and 28 patients with MPO-AAV diagnosed in Beijing Friendship Hospital,Capital Medical University from Jan 1st,2017 to Jan 1st,2022.Chest high-resolution CT(HRCT)showed usual interstitial pneumonia(UIP)or probable UIP in both groups of subjects.The demographics,clinical manifestations,laboratory tests,results of blood gas analysis and pulmonary function tests of the two groups were retrospectively analyzed.Results The age,smoking index,proportions of cough and expectoration in IPF group were higher than those in MPO-AAV group,and the proportions of fever,weight loss and joint pain in MPO-AAV group were higher than those in IPF group;compared with IPF group,MPO-AAV group had significantly higher white blood cells and percentage of neutrophils,lower hemoglobin,higher platelet,higher C-reactive protein,higher erythrocyte sedimentation rate,higher blood urea nitrogen,higher creatinine,and higher urinary occult blood and urinary protein positive rate.The levels of lactic dehydrogenase,carcinoembryonic antigen and cytokeratin 19 fragment in IPF group were significantly higher than those in MPO-AAV group.MPO-AAV group had significantly higher arterial blood pH,lower partial pressure of carbon dioxide and bicarbonate compared with IPF group.Forced vital capacity and diffusing capacity for carbon monoxide percent predicted in IPF group were lower than those in MPO-AAV group,all the above differences were statistically significant(P<0.05).Conclusion The possibility of MPO-AAV should be considered in patients with UIP or probable UIP on chest HRCT if the patients are with systemic symptoms(fever,weight loss,joint pain),high ESR,anemia and renal damage.Patients who have already been diagnosed with IPF should also be retested autoantibodies to recognize MPO-AAV if the above manifestations occur during follow-up.