Department of Internal Medicine, Toho University Medical Center Sakura Hospital, Sakura, Japan
Department of Internal Medicine, Toho University Medical Center Sakura Hospital, Sakura, Japan.
BMJ Case Rep. 2021 Jul 2;14(7):e242901. doi: 10.1136/bcr-2021-242901.
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown aetiology that causes recurrent episodes of diffuse alveolar haemorrhage (DAH). A male patient in his 50s had repeatedly experienced hemoptysis for the past 6 years, along with a decrease in the pulmonary diffusing capacity and chronic respiratory failure. After a 6-year follow-up, the patient experienced sudden exacerbation of hemoptysis and respiratory failure, and he was hospitalised. A CT of the chest revealed diffuse pulmonary infiltrates, whereas the bronchoalveolar lavage revealed hemosiderin-laden macrophages. Thus, the patient was diagnosed with DAH. As all diseases that cause DAH other than IPH were negative, the patient was suspected of IPH. He was treated with a combination of glucocorticoids and azathioprine, and his hemoptysis and chronic respiratory failure improved; however, the decrease in the pulmonary diffusing capacity did not improve. Treating adult-onset IPH with glucocorticoids and azathioprine might not improve pulmonary diffusing capacity.
特发性肺含铁血黄素沉着症（IPH）是一种病因不明的罕见疾病，可导致弥漫性肺泡出血（DAH）反复发作。一位 50 多岁的男性患者在过去 6 年中反复出现咯血，同时伴有肺弥散功能下降和慢性呼吸衰竭。经过 6 年的随访，患者出现咯血和呼吸衰竭突然加重，并住院治疗。胸部 CT 显示弥漫性肺部浸润，而支气管肺泡灌洗显示含铁血黄素的巨噬细胞。因此，患者被诊断为 DAH。由于所有引起 DAH 的疾病均为阴性，除了 IPH，因此怀疑患者患有 IPH。他接受了糖皮质激素和硫唑嘌呤的联合治疗，咯血和慢性呼吸衰竭得到改善；然而，肺弥散功能的下降并未改善。用糖皮质激素和硫唑嘌呤治疗成人发病的 IPH 可能不会改善肺弥散功能。
