Mu Xiang Dong, Su Li, Nie Li Gong, Na Jia, Wang Ren Gui, Li Hai Chao
Department of Respiratory Medicine, Peking University First Hospital, Beijing, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2008 Dec 18;40(6):595-9.
To describe the clinical, radiological and pathological characteristics of idiopathic pulmonary haemosiderosis (IPH) in adults and to evaluate the methods of diagnosis and treatment.
Two patients were successfully diagnosed and treated in our hospital and the literature on the subject was reviewed.
Two adult patients (19 and 34 years old) diagnosed in our hospital had 5 and 10 years of history of hemoptysis respectively, and chest CT showed bilateral diffuse alveolar opacities over mid and lower zones. Tests of antinuclear antibodies (ANAs), rheumatoid factor (RF), antineutrophilic cytopasmic antibodies (ANCA) and Anti-glomerular basement membrane (anti-GBM) antibody were negative. Haemosiderin-laden macrophages were found in bronchoalveolar lavage fluid (BALF) whose color was yellow. Microscopic examination of the lung tissue specimens obtained by transbronchial lung biopsy (TBLB) revealed hemorrhage and numerous hemosiderin-laden macrophages in the alveoli and no vasculitis or capillaritis were seen. These findings were consistent with a diagnosis of IPH. Steroid therapy had good effects.
IPH is a diagnosis of exclusion of other causes of diffuse alveolar hemorrhage (DAH). IPH adults have relatively good drug responses and relatively good prognoses.
描述成人特发性肺含铁血黄素沉着症（IPH）的临床、影像学及病理特征，并评估其诊断和治疗方法。
我院成功诊断并治疗了2例患者，并对该主题的文献进行了回顾。
我院诊断的2例成年患者（分别为19岁和34岁）咯血病史分别为5年和10年，胸部CT显示双侧中下肺野弥漫性肺泡实变。抗核抗体（ANA）、类风湿因子（RF）、抗中性粒细胞胞浆抗体（ANCA）及抗肾小球基底膜（anti-GBM）抗体检测均为阴性。支气管肺泡灌洗液（BALF）呈黄色，可见含铁血黄素巨噬细胞。经支气管肺活检（TBLB）获取的肺组织标本显微镜检查显示肺泡内出血及大量含铁血黄素巨噬细胞，未见血管炎或毛细血管炎。这些发现符合IPH的诊断。激素治疗效果良好。
IPH是排除其他弥漫性肺泡出血（DAH）原因后的诊断。成年IPH患者药物反应相对良好，预后相对较好。
