New York Institute of Technology College of Osteopathic Medicine at Arkansas State University, Jonesboro, AR, USA.
Division of Internal Medicine, Bangladesh Medical College, Dhaka, Bangladesh.
Am J Med Sci. 2022 Jul;364(1):111-117. doi: 10.1016/j.amjms.2021.10.030. Epub 2022 Mar 8.
Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of recurrent episodes of diffuse alveolar hemorrhage (DAH). IPH commonly manifests with hemoptysis, radiologic chest infiltrates and anemia. The etiology of IPH is unknown, but an immunologic mechanism is widely speculated. The definitive diagnosis of IPH requires a thorough exclusion of other causes of DAH, such as infections, inflammation, malignancy, cardiac diseases, drug and toxin exposure, and medications. Due to the rarity of the disease, a diagnosis is often delayed by years. We present the case of a 49-year-old man with ischemic cardiomyopathy who presented with hemoptysis for eighteen months. Serologic workup was negative for vasculitides and autoimmune diseases. Bronchoscopy revealed DAH. A surgical lung biopsy showed 'bland pulmonary hemorrhage.' A right heart catheterization ruled out cardiac causes of DAH. The patient was diagnosed with IPH and started on systemic corticosteroids with rapid improvement of hemoptysis.
特发性肺含铁血黄素沉着症（IPH）是弥漫性肺泡出血（DAH）反复发作的罕见原因。IPH 常表现为咯血、胸部影像学浸润和贫血。IPH 的病因不明，但广泛推测是一种免疫机制。IPH 的明确诊断需要彻底排除 DAH 的其他原因，如感染、炎症、恶性肿瘤、心脏病、药物和毒素暴露以及药物。由于该疾病罕见，因此通常要延迟数年才能做出诊断。我们报告了一例 49 岁男性患有缺血性心肌病，出现咯血 18 个月。血清学检查排除了血管炎和自身免疫性疾病。支气管镜检查显示 DAH。肺活检显示“温和性肺出血”。右心导管检查排除了 DAH 的心脏原因。该患者被诊断为 IPH，并开始全身用皮质类固醇治疗，咯血迅速改善。
