儿童弥漫性肺泡出血

Diffuse alveolar hemorrhage in children

摘要:

弥漫性肺泡出血（DAH）是以咯血、贫血、肺部弥漫性浸润影为主要表现的临床综合征，急性期死亡率高，是危及生命的急重症。与成人相比较，儿童DHA的基础疾病谱有其自身特点。既往认为儿童DAH以特发性肺含铁血黄素沉着症为主，但随着儿童DAH病例报道的增多，其病因谱在不断扩大。不同病因导致的DAH治疗及预后不尽相同，该文从广义角度评述儿童DAH，重点讨论特发性肺含铁血黄素沉着症、系统性红斑狼疮、抗中性粒细胞胞浆抗体相关性血管炎、COPA综合征、IgA血管炎引起的DAH。

Abstract:

Diffuse alveolar hemorrhage (DAH) is a clinical syndrome with major clinical manifestations of hemoptysis, anemia, and diffuse infiltration in the lung. DAH has a high mortality rate in the acute stage and is a life-threatening emergency in clinical practice. Compared with adult DHA, childhood DHA tends to have a specific spectrum of underlying diseases. It has long been believed that idiopathic pulmonary hemosiderosis (IPH) is the main cause of childhood DAH; however, with the increase in reports of childhood DAH cases, the etiology spectrum of childhood DAH is expanding. The treatment and prognosis of DAH with different etiologies are different. This review article gives a general outline of childhood DAH, with focuses on DAH caused by IPH, systemic lupus erythematosus, anti-neutrophil cytoplasmic antibody-related vasculitis, COPA syndrome, or IgA vasculitis.