抗中性粒细胞胞质抗体相关性血管炎合并间质性肺疾病的临床特征与预后

Clinical Characteristics and Prognosis of Antineutrophil Cytoplasmic Antibody Associated Vasculitis with Interstitial Lung Disease

目的分析抗中性粒细胞胞质抗体相关性血管炎(AAV)合并间质性肺疾病(ILD)的临床特征和预后。方法收集2014年1月至2018年12月就诊于郑州大学第一附属医院的356例AAV患者临床资料作为研究对象。根据胸部CT结果分为AAV合并ILD组(n=109)和单纯AAV组(n=247),比较两组患者的临床特征与预后。结果AAV合并ILD组平均年龄大于单纯AAV组,咳嗽、胸闷发生率,抗髓过氧化物酶抗体(MPO-ANCA)、抗蛋白酶3抗体(PR3-ANCA)阳性率高于单纯AAV组,差异有统计学意义(P<0.05)。AAV合并ILD组中显微镜下多血管炎(MPA)患者少于单纯AAV组,差异有统计学意义(P<0.05)。两组性别、吸烟人数、伯明翰系统性血管炎活动性评分(BVAS)比较,差异无统计学意义(P>0.05)。AAV合并ILD组耳鼻喉累及率高于单纯AAV组,差异有统计学意义(P<0.05);两组其他器官累及率比较,差异无统计学意义(P>0.05)。356例AAV患者中,306例(86.00%)完成随访,中位随访时间[31(19,56)]个月。90例AAV合并ILD患者中43例死亡,216例单纯AAV患者中51例死亡。AAV合并ILD组患者生存率低于单纯AAV组,差异有统计学意义(P<0.05)。结论与单纯AAV相比,AAV合并ILD组患者年龄大,MPO-ANCA阳性率高,PR3-ANCA阳性率低,更易有咳嗽、胸闷等症状,预后较差。

Objective To analyze the clinical features and prognosis of antineutrophil cytoplasmic antibody associated vasculitis(AAV)with interstitial lung disease(ILD).Methods The clinical data of 356 AAV patients in the First Affiliated Hospital of Zhengzhou University from January 2014 to December 2018 were collected.They were divided into AAV with ILD group(n=109)and simple AAV group(n=247)according to the results of chest CT.The clinical characteristics and prognosis of the two groups were compared.Results The average age of AAV combined with ILD group was higher than that of simple AAV group,the incidence of cough,chest tightness,MPO-ANCA,PR3-ANCA positive rate was higher than that of simple AAV group,the differences were statistically significant(P<0.05).The number of microscopic polyangitis(MPA)patients in AAV combined with ILD group was less than that in AAV group(P<0.05).There were no significant differences in gender,number of smokers and Birmingham systemic vasculitis activity score(BVAS)between the two groups(P>0.05).The incidence of otolaryngology involvement in AAV combined with ILD group was higher than that in AAV group(P<0.05).There was no significant difference in the incidence of other organs involvement between the two groups(P>0.05).Of the 356 AAV patients,306(86.00%)completed the follow-up,with a median follow-up time of[31(19,56)]months.Forty three of 90 patients with AAV and ILD died,and 51 of 216 patients with AAV alone died.The survival rate of AAV combined with ILD group was lower than that of AAV group(P<0.05).Conclusion Compared with simple AAV,AAV combined with ILD patients are older,higher positive rate of MPO-ANCA,lower positive rate of PR3-ANCA,more prone to cough,chest tightness and other symptoms,with poor prognosis.