Abstract: Background　Granulomatous polyangiitis（GPA）is a rare systematic disease that mainly affects the upper respiratory tract，lung and kidney.Diffuse alveolar hemorrhage（DAH）is a rare manifestation of the disease.Once it occurs，the situation is critical with a high mortality rate.Objective　To investigate the clinical manifestations，laboratory and imaging features，diagnosis and treatment as well as prognosis of GPA with DAH，enhancing clinicians' understanding of this disease to perform a diagnosis and deliver appropriate treatment early to improve the patient prognosis.Methods　The clinical data of a GPA patient with DAH admitted to Department of Rhrumatology and Immunology，Ruijin Hospital，Shanghai Jiao Tong University School of Medicine，was reviewed and analyzed.Besides，publicly published reports on GPA or GPA with DAH during January 1980 to February 2019 were retrieved from electronic databases of Wanfang Data Knowledge Service Platform，CNKI，and PubMed using "Wegener，vasculitis，granulomatous polyangiitis，pneumorrhagia，and alveolar hemorrhage" as keywords.Results　12 cases were reported，combined with our case，there were 13 cases of GPA with DAH，including 7 males and 6 females，with an average age of（38.6±19.3）years，and an average duration of（72.1±62.7）days.Most of them were treated with high-dose intravenous corticosteroid pulse therapy combined with cyclophosphamide.The rate of mortality was 23.1%（3/13）.Commonly seen clinical manifestations were cough（83.3%），dyspnea（83.3%），and hemoptysis（42.7%）.Chest X-rays or CT scans showed multiple patches，ground-glass opacities or bilateral diffuse alveolar infiltrates and consolidations.Bronchoscopy may show airway hemorrhage，and elevated number of hemosiderin-laden macrophages could be found in bronchoalveolar lavage fluid.Conclusion　DAH is a rare manifestation of GPA，characterized mainly by dyspnea，cough，hemoptysis，rapid progress，and high mortality rate.Early treatment may improve the prognosis.

Key words: Vasculitis, Granuloma, Granulomatous polyangiitis, Hemorrhage, Pulmonary alveoli, Diffuse alveolar hemorrhage, Case reports

摘要： 背景　肉芽肿性多血管炎（GPA）是一种少见的全身性疾病，主要影响上呼吸道、肺部和肾脏，弥漫性肺泡出血（DAH）是该病的罕见表现，一旦出现，病情危重，死亡率高。目的　探讨GPA合并DAH的临床表现、影像学检查和辅助检查的特征、诊治及预后，以期提高对该病的认识，及早诊断并改善预后。方法　分析上海交通大学医学院附属瑞金医院风湿免疫科收治的1例GPA合并DAH患者的临床资料，并以“韦格纳，血管炎，肉芽肿性多血管炎，肺出血，肺泡出血”为关键字，搜索万方数据知识服务平台、中国知网、PubMed数据库1980年1月—2019年2月公开发表的GPA/韦格纳肉芽肿（WG）合并DAH的病例。结果　共检索到国内外报道病例12例，结合本例，共纳入13例患者，其中男7例，女6例；平均年龄（38.6±19.3）岁；平均病程（72.1±62.7）d；治疗多选择大剂量激素冲击治疗和环磷酰胺（CTX）治疗；死亡率23.1%（3/13）。临床常见咳嗽（83.3%）、呼吸困难（83.3%）、咯血（41.7%）等。胸部X线片或胸部CT见多发斑片状、磨玻璃影或弥漫性肺部浸润影、实变影。支气管镜和肺泡灌洗液可见出血及含有含铁血黄色的巨噬细胞。结论　DAH是GPA较少见的表现，多表现为呼吸困难、咳嗽、咯血等，病情进展快、死亡率高，及早治疗可改善预后。

关键词: 血管炎, 肉芽肿, 肉芽肿性多血管炎, 出血, 肺泡, 弥漫性肺泡出血, 病例报告