成人特发性肺含铁血黄素沉着症7例并文献复习

摘要

目的 探讨成人特发性肺含铁血黄素沉着症(idiopathic pulmonary hemosiderosis,IPH)的主要临床特点、诊断、治疗及预后.方法 收集近10年我院收治的成人IPH患者,总结其临床特点、治疗方法及预后,并结合国内外相关报道进行文献分析.结果 近10年共诊断7例成人IPH患者,男4例,女3例,年龄14～41岁,平均24岁.主要临床表现为发热(6/7)、咯血(5/7)、呼吸困难(5/7).主要体征有肺部啰音(3/7)、肝脾肿大(2/7).主要胸部影像学表现为磨玻璃影(7/7)、实变影(3/7)、气胸影(1/7).7例患者住院期间均应用糖皮质激素治疗,其中3例患者因呼吸衰竭行糖皮质激素冲击及机械通气治疗,所有患者均病情好转后出院,随访l～74个月,均存活.结论 成人IPH主要特点为不明原因的咯血、肺部浸润影及贫血,对糖皮质激素治疗反应较好,预后相对较好,与文献报道一致.%Objective To analyze the main clinical feature,diagnosis,treatment and prognosis of adult idiopathic pulmonary hemosiderosis (IPH).Methods The clinical data of adult IPH patients treated in our hospital in the near ten years were collected,reviewed and analyzed together with domestic and abroad literature.Results Seven cases of adult IPH patients were diagnosed,4 male and 3 female,aged from 14 to 41,mean 24 years.The main clinical manifestations included fever(6/7),hemoptysis(5/7)and dyspnea(5/7).The main signs were rale (3/7) and hepatosplenomegaly (2/7).The main image demonstrations were ground-glass opacity (7/7),consolidation (3/7) and pneumothorax (1/7).All the patients received glucocorticoids treatment,3 of them received glucocorticoids stoss therapy and mechanical ventilation.All patients were improved and discharged from hospital.All patients were followed up and alive.The follow-up time was different from 1 to 74 months.Conclusions Adult IPH is characterized by hemoptysis,pulmonary infiltrates and anemia of unknown etiology.Good response to glucocorticoids treatment and a relatively good prognosis were seen in adult IPH cases and that were coincided with literature.