目的探讨儿童特发性肺含铁血黄素沉着症(IPH)的误诊情况。方法对临床上被误诊的23例IPH进行回顾性分析。结果误诊为肺炎10例,肺结核4例,缺铁性贫血4例,溶血性贫血4例,支气管扩张1例,慢性消化道失血1例。确诊后均糖皮质激素治疗,5例联合硫唑嘌呤,随访1~3 a,不发作9例,发作12例,死亡1例,失访1例。结论 IPH临床表现多样化,临床医师应提高对该病的认识减少误诊。 Objective To investigate the misdiagnosis of idiopathic pulmonary hemosiderosis (IPH) in children. Methods The clinical data of 23 cases misdiagnosed as IPH were analyzed retrospectively. Results Misdiagnosed as pneumonia in 10 cases, 4 cases of pulmonary tuberculosis, iron deficiency anemia in 4 cases, 4 cases of hemolytic anemia, bronchiectasis in 1 case, 1 case of chronic gastrointestinal bleeding. All the patients were treated with glucocorticoid after the diagnosis. Five cases were united with azathioprine. The patients were followed up for 1-3 years without any episodes, 9 cases were attacked, 12 cases were attacked, 1 case died and 1 case was lost. Conclusions The clinical manifestation of IPH is diversified. Clinicians should improve the understanding of the disease to reduce misdiagnosis.