9例抗中性粒细胞胞浆抗体相关性血管炎肺出血临床分析

Clinical analysis of 9 cases of antineutrophil cytoplasmic antibody associated vasculitis pulmonary hemorrhage

目的:回顾性分析抗中性粒细胞胞浆抗体相关性血管炎(AAV)合并弥漫性肺泡出血的临床表现特点、治疗及转归情况。方法:收集2010年1月至2020年1月,于首都医科大学附属北京友谊医院AAV合并弥漫性肺泡出血患者9例,分析其临床症状、实验室检查、胸部影像学表现、治疗及转归。结果:AVV合并肺出血患者中临床表现为咯血7例(77.8%),咳嗽8例(88.9%),咳痰7例(77.8%),呼吸困难2例(22.2%),发热3例(33.3%)及乏力1例(11.1%)。9例患者均出现不同程度贫血,3例患者血清肌酐升高,4例患者出现蛋白尿,5例出现血尿,核周型中性粒细胞胞浆抗体(P-ANCA)阳性9例,抗髓过氧化物酶IgG(MPO-IgG)阳性6例。胸部影像学主要表现为肺实变、磨玻璃影及斑片渗出影,9例患者合并肺部感染,1例出现胸腔积液。采用甲强龙冲击3例,联合环磷酰胺药物治疗3例。2例因呼吸衰竭死亡,病死率为22.2%。结论:AAV合并弥漫性肺泡出血,病情进展极快,临床症状多样,病死率较高,治疗时需严密监测实验室指标、影像学改变,对判断病情进展有重要作用。

Objective:To analyze the clinical characteristics,treatment and outcome of anti-neutrophil cytoplasmic antibody-related vasculitis(AAV)combined with diffuse alveolar hemorrhage.Methods:From January 2010 to January 2020,9 patients with AAV combined with diffuse alveolar hemorrhage in Beijing Friendship Hospital were collected,and clinical symptoms,laboratory examinations,chest imaging findings,treatment and outcome were analyzed.Results:The clinical manifestations of AVV patients with pulmonary hemorrhage included hemoptysis in 7 cases(77.8%),cough in 8 cases(88.9%),sputum in 7 cases(77.8%),dyspnea in 2 cases(22.2%),and fever in 3 cases(33.3)%and 1 case of fatigue(11.1%).Nine patients all developed anemia,3 patients with elevated serum creatinine,and 9 patients with perinuclear neutrophil cytoplasmic antibody(P-ANCA)positive.3 cases were treated with corticosteroids and 3 cases were treated with cyclophosphamide.Conclusions:AAV combined with diffuse alveolar hemorrhage,the disease pro-gresses very quickly,and the mortality rate is high.During treatment,laboratory indicators and imaging changes must be monitored,which plays an important role in judging the progress of the disease.