[成人特发性肺含铁血黄素沉着症]

[Idiopathic pulmonary hemosiderosis in adults].

作者信息

Maalej S, Drira I, Fennira H, Ben Mefteh R, Bourguiba M, Zidi A, El Mezni F, Ben Kheder A

机构信息

Service de Pneumologie, Hôpital de Pneumo-Phtisiologie Abderrahman-Mami, 2080 L'Ariana/Tunis, Tunisia.

出版信息

Rev Pneumol Clin. 2005 Apr;61(2):109-11. doi: 10.1016/s0761-8417(05)84796-0.

DOI:10.1016/s0761-8417(05)84796-0

PMID:16012364

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage characterized by abnormal deposition of hemosiderin iron in the macrophages of alveoli. Most cases occur in children. In adults, IPH is rare: almost 10 cases reported during the late 10 years. We report the case of a 20-year-old-girl with IPH. There was no evidence of pulmonary vasculitis or capillaritis. Long-term treatment with systemic corticosteroids was followed by clinical remission lasting 4 years.

摘要

特发性肺含铁血黄素沉着症（IPH）是弥漫性肺泡出血的一种罕见病因，其特征为含铁血黄素铁在肺泡巨噬细胞中异常沉积。大多数病例发生在儿童。在成人中，IPH很罕见：近10年来报告了约10例。我们报告一例20岁患IPH的女孩。没有肺血管炎或毛细血管炎的证据。长期使用全身性皮质类固醇治疗后临床缓解持续了4年。