Germouty J, Bonnaud F, Rouffaud J
Sem Hop. 1976 Feb 9;52(6):369-75.
Idiopathic pulmonary hemosiderosis gives rise to anemia, due to repeated intra-alveolar hemorrhage, the reabsorption of which leads to hemosiderin deposits in the lung parenchyma. The authors report a case in a young woman aged 24 years whose illness started with anemia, then two months later, with hemoptysis and a broncho-pulmonary syndrome with a low grade fever. On the 6th month, there occurred a hazy infiltrate of both lung bases which was fleeting, mobile and recurrent. In the light of this triad of anemia, hemoptysis and infiltrates, the diagnosis of idiopathic pulmonary hemosiderosis was made and confirmed by three examinations:--Lung biopsy: siderophages were found in the sub-mucosa,--Radio-isotope examination, using Fe 59 which revealed iron deposits in the lung,--A surgical lung biopsy which showed a congestive area and a fibrous area. The congestive area was the site of recent hemorrhage, the alveolar limits were filled with siderophages. The fibrous area was the site of chronic repair of older hemorrhage. It was mutilating. The course was complicated by massive bleeding which led to acute recovering respiratory failure. The patient is at present stabilised by corticosteroids. Three hundred cases of idiopathic pulmonary hemosiderosis have been reported in the world literature. Although the main characteristic is intra-alveolar hemorrhage, its course has not yet been determined. It seems however, to be due to an immunologic process as shown by the relationship between this curious disease and Goodpasture's syndrome.
特发性肺含铁血黄素沉着症可导致贫血，这是由于反复的肺泡内出血，出血的再吸收导致含铁血黄素沉积于肺实质。作者报告了一例24岁年轻女性病例，其疾病始于贫血，两个月后出现咯血及伴有低热的支气管-肺综合征。在第6个月时，双肺底部出现模糊浸润影，呈一过性、游走性且反复出现。鉴于贫血、咯血和浸润影这三联征，诊断为特发性肺含铁血黄素沉着症，并通过三项检查得以证实：——肺活检：在黏膜下层发现噬铁细胞；——放射性同位素检查，使用铁59显示肺部有铁沉积；——外科肺活检显示有充血区和纤维区。充血区是近期出血部位，肺泡界限处充满噬铁细胞。纤维区是陈旧性出血慢性修复部位。这是致残性的。病程因大量出血而复杂化，导致急性呼吸衰竭恢复。患者目前通过皮质类固醇治疗病情稳定。世界文献中已报道了300例特发性肺含铁血黄素沉着症病例。尽管其主要特征是肺泡内出血，但其病程尚未明确。然而，从这种奇特疾病与古德帕斯丘综合征的关系来看，似乎是由免疫过程引起的。
