摘要
目的总结抗中性粒细胞胞浆抗体(ANCA)相关性血管炎导致的弥漫性肺泡出血(DAH)的临床表现、影像学特点、诊治经验及预后, 以提高对该病的认识。方法回顾性分析南京大学医学院附属鼓楼医院急诊科收治的2例ANCA相关性血管炎合并DAH患者的临床资料, 并以"血管炎""ANCA相关性血管炎""弥漫性肺泡出血"和"肺泡出血"为关键词, 检索万方医学数据、中国知网、PubMed、Embase数据库1980年1月至2021年8月公开发表的病例报道。结果南京大学医学院附属鼓楼医院急诊科收治的2例患者病情危重均需ECMO支持治疗, 同时联合俯卧位通气有助于病情缓解, 经治疗均好转出院。文献复习共检索到符合标准的文章29篇, 患者32例, 结合我科2例, 共34例患者纳入分析。其中男16例, 女18例;年龄45.5(28.0, 73.25)岁, 年龄范围为8~81岁;病程13(8, 20) d, 病程范围为2~85 d, 总体病死率20.59%(7/34)。临床表现以咳嗽、咯血、呼吸困难等多见, 严重的可出现急性呼吸窘迫综合征、急性肾损伤。胸部影像学可见多发斑片状、弥漫性肺部浸润或实变影。支气管镜和肺泡灌洗液可见出血及含有含铁血黄素的巨噬细胞。治疗多选择大剂量激素冲击和环磷酰胺的免疫抑制疗法, ECMO是重要的挽救性治疗措施(35.29%, 12/34), 使用ECMO支持治疗的患者无1例死亡。结论 DAH是ANCA相关性血管炎的罕见临床表现, 预示病情危重。早期应用ECMO联合俯卧位通气有利于分泌物引流、促进肺复张, 是重要的支持治疗手段, 可为后期病因诊治争取宝贵时间。
ObjectiveTo summarize the clinical manifestations, imaging features, the experience of diagnosis and treatment, and prognosis of diffuse alveolar hemorrhage (DAH) caused by anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).MethodsThe clinical data of the 2 patients with AAV complicated with DAH admitted to the emergency department of the Affiliated Drum Tower Hospital of Nanjing University Medical School were analyzed retrospectively." Vasculitis" , " ANCA-associated vasculitis" , " diffuse alveolar hemorrhage" and " alveolar hemorrhage" were used as keywords.The case reports published by Wanfang Medical database, CNKI, PubMed and Embase database from January 1980 to August 2021 were searched.ResultsBoth critically ill patients needed ECMO support.After combining with prone ventilation, their conditions were alleviated.Finally, they improved articles including 32 patients meeting the standard were retrieved.With 2 patients from our department, a total of 34 patients were included in the analysis.There were 16 males and 18 females, with the median age of them 45.5 (28.0 and 73.25) years, the age range from 8 to 81 years.The median duration of disease was 13 (8 and 20) days, the course of disease from 2 to 85 days, and the overall mortality is 20.59% (7/34).The clinical features of DAH include coughing, hemoptysis and dyspnea, even progressing acute respiratory distress syndrome and acute kidney injury.Chest imaging showed multiple patchy, diffuse pulmonary infiltration or consolidation.Bronchoscopy and alveolar lavage fluid showed bleeding and macrophages containing hemosiderin.Immunosuppressive therapy with high-dose hormone shock and cyclophosphamide was selected for treatment.ECMO is an essential salvage treatment (35.29%, 12/34), and none of them who received ECMO support therapy died.ConclusionsDAH is one of the rare serious complications and the strongest predictor of early mortality in the course of AAV.ECMO combined with prone position ventilation is a feasible and efficient treatment
作者
刘瑶
张秋灵
刘恒均
王玮
何飞
徐鹏
Liu Yao;Zhang Qiuling;Liu Hengjun;Wang Wei;He Fei;Xu Peng(Department of Emergency,the Affiliated Drum Tower Hospital of Nanjing University Medical School,Nanjing 210008,China)
出处
《国际呼吸杂志》
2022年第10期752-758,共7页
International Journal of Respiration
基金
南京市卫生科技发展项目(YKK19065)。
