抗中性粒细胞胞浆抗体相关性血管炎致弥散性肺泡出血2例

弥散性肺泡出血（DAH）是一个临床综合征,可由多种病因引起,抗中性粒细胞抗体（ANCA）相关性血管炎是众多原因之一。ANCA相关性血管炎引起的DAH在儿童很少见。我们报道了两例儿童ANCA相关性血管炎引起的DAH,这两例患儿均被误诊为特发性肺含铁血黄素沉着症。一例是一个8岁女孩,被误诊为特发性肺含铁血黄素沉着症5年,最终被诊断为ANCA相关性血管炎。另一例是一个12岁的男孩,由于甲状腺功能亢进服用丙硫氧嘧啶2年,出现咳嗽、咯血和贫血,被误诊为肺含铁血黄素沉着症,该患儿病情进展迅速,最终被诊断为丙硫氧嘧啶诱发的ANCA相关性血管炎。ANCA相关性血管炎是一个系统性疾病,可累及多个脏器,当累及肺部时,在急性发作期出现弥散性肺泡出血的临床症候群,激素联合其他免疫抑制剂是其主要治疗方法。对于临床出现的DAH,明确潜在的病因是至关重要的,因为只有明确诊断,才能决定治疗及判断治疗效果。

Diffuse pulmonary alveolar hemorrhage caused by anti-neutrophil antibody associated vasculitis in two children

Diffuse alveolar hemorrhage (DAH) is a clinical syndrome that can be a manifestation of multiple different causes. antineutrophil cytoplasmic antibody (ANCA) - associated vasculitides is one cause of diffuse alveolar hemorrhage, and is a rare disease in children. We present 2 cases of ANCA- associated vasculitides which misdiagnosed with idiopathic pulmonary haemosiderosis. One was an 8-yr-old girl, who was diagnosed as IPH for about 5 years, and finally diagnosed with ANCA- associated vasculitides. Another was a 12-yr-old boy, who had been treated with propylthiouracil (PTU) for about two year because of hyperthyroidism. He had the triad of idiopathic pulmonary haemosiderosis, and progressed rapidly, at last, PTU indued ANCA- associated vasculitides was diagnosed. Glucocorticoids in combination with other immunosuppressants are main therapied for ANCA- associated vasculitides. For DAH, identification of the underlying etiology is of utmost importance and dictates treatment.